|
1
|
Suzuki K, Akiyama M, Kaneko Y. Visceral disseminated varicella zoster virus infection masquerading as lupus mesenteric vasculitis recurrence. Mod Rheumatol Case Rep 2025; 9:63-65. [PMID: 39219550 DOI: 10.1093/mrcr/rxae051] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2024] [Revised: 08/18/2024] [Accepted: 08/20/2024] [Indexed: 09/04/2024]
Affiliation(s)
- Koji Suzuki
- Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Mitsuhiro Akiyama
- Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Yuko Kaneko
- Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
| |
Collapse
|
|
2
|
Weinberg S, Amarnani A, Jolly M. Gastrointestinal and hepatic manifestations. DUBOIS' LUPUS ERYTHEMATOSUS AND RELATED SYNDROMES 2025:505-520. [DOI: 10.1016/b978-0-323-93232-5.00045-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
|
|
3
|
Almutairi R, Alkhudair D, Aldei A. Lupus Enteritis as the Early Manifestation of Systemic Lupus Erythematosus Successfully Managed With Belimumab: A Case Report. Cureus 2025; 17:e76926. [PMID: 39906430 PMCID: PMC11791376 DOI: 10.7759/cureus.76926] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/04/2025] [Indexed: 02/06/2025] Open
Abstract
Lupus enteritis (LE) is an uncommon cause of abdominal pain in individuals with systemic lupus erythematosus (SLE). This condition is explained by the inflammation of the intestinal wall caused by the accumulation of immune complexes and activation of the complement system. A 19-year-old previously healthy Kuwaiti woman arrived at the emergency department (ED) complaining of diffuse abdominal pain, non-bloody diarrhea, and nausea. The symptoms started two days before presentation. The patient described the abdominal pain as sudden, sharp, and stabbing in nature, with a severity of 10 out of 10, and mainly located in the suprapubic area. On examination, the patient's vital signs were normal. She had no skin rash, oral ulcers, arthritis, or palpable lymphadenopathy. Her abdomen was soft on palpation, with diffuse tenderness. Her initial laboratory investigations showed a normal hemoglobin level and WBC count but lymphopenia (0.6 x 109/L; normal, 1-3 x 109/L). A computed tomography (CT) scan of the abdomen showed small bowel loops with diffuse edematous wall thickening with target signs. The descending and sigmoid colon, and the rectum walls were also thickened and edematous. Significant pericolonic fat stranding and free fluid with preserved enhancement suggested an active systemic inflammatory process. Anti-nuclear antibody and anti-double stranded DNA antibody test results were positive. Acute abdominal pain was managed with IV corticosteroids, and 10 mg/kg IV belimumab was initiated. The patient was followed up in OPD after one, three, and six months, where she did not mention any relapse of LE nor any side effects from belimumab. The diagnosis of LE in this case was challenging because of the absence of a prior diagnosis of SLE. Clinical manifestations of this condition, which include abdominal discomfort, vomiting, and diarrhea, are non-specific and can be mistaken for several chronic gastrointestinal disorders, posing a diagnostic problem. Although belimumab is not commonly used to treat LE, we were able to successfully manage the patient using it, making it a promising new method for treating LE.
Collapse
Affiliation(s)
- Rawan Almutairi
- Dermatology, Farwaniya Hospital, Ministry of Health, Farwaniya, KWT
| | | | - Ali Aldei
- Rheumatology, Amiri Hospital, Kuwait City, KWT
| |
Collapse
|
|
4
|
Mehta P, Aggarwal A, Rajasekhar L, Shobha V, Mathew AJ, Kavadichanda C, Ghosh P, Das B, Rathi M, Srivastava A, Gupta R, Jain A. Gastrointestinal manifestations in systemic lupus erythematosus: data from an Indian multi-institutional inception (INSPIRE) cohort. Rheumatology (Oxford) 2025; 64:156-164. [PMID: 38059606 DOI: 10.1093/rheumatology/kead653] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2023] [Revised: 10/12/2023] [Accepted: 11/11/2023] [Indexed: 12/08/2023] Open
Abstract
OBJECTIVES To study the prevalence, correlates, and outcomes of GI manifestations in a prospectively enrolled nationwide cohort of SLE in India (INSPIRE). METHODS It is an observational cohort study with analysis of the baseline database of the INSPIRE cohort with early outcomes assessed till 10 April 2023. Cases with GI manifestations as per the BILAG index were selected, pertinent clinical and laboratory data were retrieved for analysis. Patients with GI manifestations were compared with the rest of the cohort and factors associated with death were determined. RESULTS Of the 2503 patients with SLE enrolled in the INSPIRE cohort, 243 (9.7%) had GI manifestations observed early in the disease course (1, 0-3 months). Ascites (162, 6.5%), followed by enteritis (41,1.6%), pancreatitis (35, 1.4%) and hepatitis (24, 0.9%) were the most prevalent manifestations. All patients received immunosuppressive therapy, and four patients required surgery. Twenty-nine patients died (11.9%), with uncontrolled disease activity (17, 58.6%) and infection (6, 20.7%) accounting for the majority of deaths. Low socioeconomic class [lower Hazard Ratio (95% confidence intervals, CI) 2.8 (1.1-7.9); upper lower 7.5 (2-27.7); reference as upper class] and SLEDAI 2K [1.06 (1.02-1.11)] were associated with death in the GI group. GI manifestations were significantly associated with age [odds ratio and 95% CI 0.97 (0.96-0.99)], pleural effusion [4.9 (3.6-6.7)], thrombocytopenia [1.7 (1.2-2.4)], myositis [1.7 (1.1-2.7)], albumin [0.7 (0.5-0.8)], alkaline phosphatase (ALP) [1.01 (1.0-1.002)], low C3 [1.9 (1.3-2.5)], total bilirubin [1.2 (1.03-1.3)], alopecia [0.62 (0.5-0.96], elevated anti-dsDNA [0.5 (0.4-0.8)], and anti-U1RNP antibody [0.8 (0.5-0.7)] in model one; and age [0.97 (0.96-0.99)], creatinine [1.2 (1.03-1.4)], total bilirubin [1.2 (1.03-1.3)], ALP [1.01 (1.0-1.002)], albumin [0.6 (0.5-0.7)], andanti-U1RNP antibody [0.6 (0.5-0.8)] in model two in multivariate analysis compared with patients without GI features. The mortality was higher in the GI group (11.9% and 6.6%, P = 0.01) as compared with controls. CONCLUSION GI manifestations were observed in 9.7% of the cohort and were always associated with systemic disease activity and had higher mortality.
Collapse
Affiliation(s)
- Pankti Mehta
- Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
- Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, India
| | - Amita Aggarwal
- Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Liza Rajasekhar
- Department of Clinical Immunology & Rheumatology, Nizam Institute of Medical Sciences, Hyderabad, India
| | - Vineeta Shobha
- Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, India
| | - Ashish J Mathew
- Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India
| | - Chengappa Kavadichanda
- Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry, India
| | - Parasar Ghosh
- Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education & Research, Kolkata, India
| | - Bidyut Das
- Department of Rheumatology, SCB Medical College, Cuttack, India
| | - Manish Rathi
- Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Akansha Srivastava
- Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Ranjan Gupta
- Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India
| | - Avinash Jain
- Department of Clinical Immunology and Rheumatology, SMS Medical College & Hospital, Jaipur, India
| |
Collapse
|
|
5
|
Zulfiqar F, Bilal M, Shah Y, Morgan S, Fatima R, Singh B, Sebastian SA, Roumia B, Bhatt P, Thallapally VK, Krishnamoorthy G, Hussain SAM. Navigating mesenteric vasculitis: A comprehensive review of literature. Dis Mon 2024; 70:101830. [PMID: 39592294 DOI: 10.1016/j.disamonth.2024.101830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2024]
Abstract
Vasculitides are diseases marked by inflammation of the blood vessel walls across various organ systems. The mesenteric vasculitis (MV) affects localized mesenteric vessels of the gastrointestinal tract. It usually occurs as part of a systemic inflammatory process but could also present in isolation. There are very few published reports of isolated mesenteric artery vasculitis. Presenting symptoms often include nausea, vomiting, diarrhea, abdominal pain, rectal bleeding, often complicating the diagnostic process. Diagnosing MV as the cause of abdominal pain can be challenging, and failure to diagnose can result in significant morbidity and mortality. A timely and accurate diagnosis of MV is essential for administering the appropriate immunosuppressive therapy and preventing unnecessary surgical interventions. This review aims to provide a comprehensive discussion of MV, including its clinical presentation, diagnostic approaches, and treatment options, with a focus on achieving early diagnosis to enhance outcomes and prevent complications. Furthermore, this review addresses the diagnostic challenges associated with MV, including the lack of specific criteria and symptom overlap with other gastrointestinal disorders such as atherosclerotic mesenteric ischemia, infections, malignancies, adverse medication effects, and other vessel occlusive processes. It also emphasizes the gaps in current literature regarding optimal diagnostic strategies and the necessity for standardized treatment protocols. By addressing these gaps and challenges, we aim to optimize patient care and improve prognosis for individuals affected by MV.
Collapse
Affiliation(s)
- Fizza Zulfiqar
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | - Muhammad Bilal
- Department of Internal Medicine, Bassett Medical Center, Cooperstown, NY, USA
| | - Yash Shah
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA.
| | - Steele Morgan
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | - Rida Fatima
- Department of Internal Medicine, Bassett Medical Center, Cooperstown, NY, USA
| | - Bryanna Singh
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | | | - Bashar Roumia
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | - Parjanya Bhatt
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | | | - Geetha Krishnamoorthy
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | - Syed Ali Muttaqi Hussain
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| |
Collapse
|
|
6
|
Louthrenoo W, Gumtorntip W, Thanunchai P, Amantakul A, Kasitanon N, Pojchamarnwiputh S. Clinical features, imaging findings, and outcomes of acute abdominal pain in systemic lupus erythematosus: comparing mesenteric vasculitis, non-mesenteric vasculitis, and surgical conditions. Clin Rheumatol 2024; 43:3699-3712. [PMID: 39422805 DOI: 10.1007/s10067-024-07189-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2024] [Revised: 09/14/2024] [Accepted: 10/11/2024] [Indexed: 10/19/2024]
Abstract
OBJECTIVES To determine the spectrum, clinical features and outcomes in systemic lupus erythematosus (SLE) patients with acute abdominal pain (AAP). METHOD Medical records of SLE patients in a lupus cohort from January 1987 to June 2023 were reviewed. Patients with AAP requiring hospitalization were identified and categorized into 3 groups: lupus mesenteric vasculitis (LMV), non-LMV, and surgical AAP. Each AAP episode represented one patient. RESULTS Of 1,538 patients in the cohort, 62 (4.03%) had 93 episodes of AAP. After exclusion, 31 patients had 39 LMV episodes, and 30 had 40 non-LMV episodes (19 due to surgical AAP). Seventy-six of the 79 AAP episodes (96.20%) were in females, with a mean ± SD age and median (IQR) disease duration of 36.76 ± 13.60 years and 6 (2, 9) years, respectively. Patients in the LMV group had more fever, nausea and vomiting, and diarrhea than those in the non-LMV group. They also had more small bowel involvement, bowel wall thickening, target water enhancement signs, mesenteric vessels engorgement and mesenteric fat cloudiness, and higher SLE disease activity. These differences were more pronounced when compared to the surgical AAP group. Treatment with corticosteroids and immunosuppressive drugs gave favorable outcomes in the LMV group. Two of 40 (5.00%) non-LMV AAP patients died, of which 1 (5.26%) was in the surgical AAP group. CONCLUSION LMV was common among SLE patients admitted for AAP. LMV usually presented with fever, gastrointestinal dysmotility symptoms, diffused abdominal pain, together with evidence of active disease. Localized abdominal pain with peritoneal signs favored surgical AAP. Key Points • Lupus mesenteric vasculitis is common among SLE patients presenting with acute abdominal pain. Its presence often associates with gastrointestinal symptoms together with other clinical manifestations of SLE • The signs in abdominal computed tomography findings are not specific and could be observed in other causes of abdominal pain in SLE. Interpretation of these signs should be cautionary and accompanied by history taking and physical abdominal findings • Treatment of lupus mesenteric vasculitis with corticosteroids alone, or in combination with immunosuppressive drugs, usually results in good outcomes.
Collapse
Affiliation(s)
- Worawit Louthrenoo
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
| | - Wanitcha Gumtorntip
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Piyanut Thanunchai
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Amonlaya Amantakul
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Nuntana Kasitanon
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Suwalee Pojchamarnwiputh
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| |
Collapse
|
|
7
|
Zhou L, Cai SZ, Dong LL. Recent advances in pathogenesis, diagnosis, and therapeutic approaches for digestive system involvement in systemic lupus erythematosus. J Dig Dis 2024; 25:410-423. [PMID: 39317429 DOI: 10.1111/1751-2980.13307] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2023] [Revised: 07/30/2024] [Accepted: 08/01/2024] [Indexed: 09/26/2024]
Abstract
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the presence of large amounts of autoantibodies and immune complex formation. Because of their atypical clinical symptoms, SLE patients with digestive system involvement may not be recognized or treated precisely and extensively. Clinicians should pay close attention to SLE with digestive system involvement, as these conditions can easily worsen the condition and possibly endanger the patient's life. In this review we summarized the pathogenesis, pathological characteristics, clinical manifestations, diagnosis, and therapies for digestive system involvement in SLE.
Collapse
Affiliation(s)
- Liang Zhou
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| | - Shao Zhe Cai
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| | - Ling Li Dong
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| |
Collapse
|
|
8
|
Cheng J, Wu Q, Wu Q, Peng Y. Lupus mesenteric vasculitis with gastrointestinal bleeding as the primary manifestation: a case report. Br J Hosp Med (Lond) 2024; 85:1-7. [PMID: 38941968 DOI: 10.12968/hmed.2024.0108] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/30/2024]
Abstract
Systemic lupus erythematosus (SLE) is a complex autoimmune disease where the body loses tolerance to its own antigens, particularly nuclear antigens. Abnormal responses from T and B cells lead to the production of autoantibodies and the formation of immune complexes in tissues, triggering complement activation, inflammation, and irreversible organ damage. SLE can affect any part of the body, resulting in diverse clinical symptoms. One rare manifestation of SLE is lupus mesenteric vasculitis (LMV), which presents with vague symptoms, abnormal laboratory findings, and specific imaging features. LMV, although uncommon, can progress to severe complications such as bowel perforation, haemorrhage, and even mortality. Here, we report a case of LMV with the involvement of multiple organ systems (including mucocutaneous, musculoskeletal, serosal cavities, and haematological systems), presenting initially with life-threatening intractable gastrointestinal bleeding, and complicated by severe pulmonary infection. By sharing this case, we aim to enhance clinicians' confidence in managing critical SLE cases and raise awareness about disease surveillance.
Collapse
Affiliation(s)
- Jirong Cheng
- Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China
| | - Qian Wu
- Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China
| | - Qiurong Wu
- Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China
| | - Yuanhong Peng
- Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China
| |
Collapse
|
|
9
|
Muñoz-Urbano M, Sangle S, D'Cruz DP. Lupus enteritis: a narrative review. Rheumatology (Oxford) 2024; 63:1494-1501. [PMID: 38216993 DOI: 10.1093/rheumatology/kead689] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Revised: 10/20/2023] [Accepted: 12/02/2023] [Indexed: 01/14/2024] Open
Abstract
Lupus enteritis (LE) is a rare manifestation of systemic lupus erythematosus. The pathophysiology of LE has not been fully elucidated, although inflammatory and thrombotic processes are likely important factors. The underlying pathophysiological mechanisms may depend on which portion of the intestine is affected. Over half of the patients with LE also present with renal or haematological complications. The diagnosis of LE is based on clinical, histopathological and imaging findings; abdominal computed tomography (CT) is the gold standard in diagnosis. Abdominal CT can also identify factors that predict complications and could potentially guide pharmacological and nutritional management. Timely identification and prompt treatment initiation are paramount to avoid life and organ threatening complications. Glucocorticoids are often the first-line treatment. Additional therapy including immunosuppressive therapy is utilised on a case-by-case basis as there are no clinical trials to define the optimal therapeutic approach. Surgical intervention may be needed especially if there is bowel perforation or peritonitis. In general, the prognosis of LE is good.
Collapse
Affiliation(s)
| | - Shirish Sangle
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - David P D'Cruz
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| |
Collapse
|
|
10
|
Preet K, Mittal BR, Singh H, Kumar R, Sekar A, Kurdia K. 18 F-FDG PET/CT in Lupus Enteritis Presenting With Fever and Abdominal Pain. Clin Nucl Med 2024; 49:e211-e212. [PMID: 38537204 DOI: 10.1097/rlu.0000000000005153] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/06/2024]
Abstract
ABSTRACT Systemic lupus erythematosus is a systemic autoimmune disease associated with various manifestations. Here, we report a compelling case of a 42-year-old woman who presented with lupus enteritis as a sole manifestation of systemic lupus erythematosus and underwent 18 F-FDG PET/CT. The resected bowel segment revealed vasculitis, and subsequent workup revealed positive antinuclear and anti-double-stranded antibody levels, confirming lupus enteritis, thus highlighting the diagnostic role of 18 F-FDG PET/CT in reaching the final diagnosis.
Collapse
Affiliation(s)
| | | | | | | | | | - Kailash Kurdia
- General Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| |
Collapse
|
|
11
|
Lourenço MH, Silva AB, Sousa J, Oliveira H, Silva I, Costa M, Branco JC, Gonçalves MJ. Necrotizing mesenteric vasculitis in systemic lupus erythematosus. ARP RHEUMATOLOGY 2024; 3:49-52. [PMID: 38558064 DOI: 10.63032/xvzp4159] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/12/2024]
Abstract
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder which may affect the gastrointestinal system. Half of the patients with SLE experience gastrointestinal symptoms, with the most common being nausea, vomiting, anorexia, and abdominal pain. Mesenteric vasculitis is a severe and rare complication of SLE and one of the most frequent causes of severe acute abdominal pain. The authors present a case of a 57-year-old woman with SLE who was diagnosed with necrotizing mesenteric vasculitis following a urinary septic shock. The patient was treated with high-dose corticosteroid therapy and cyclophosphamide, with resolution of the clinical picture.
Collapse
Affiliation(s)
| | | | - Jessica Sousa
- Radiology, Centro Hospitalar Universitário de Santo António
| | - Helena Oliveira
- Pathological Anatomy, Hospital de Cascais Dr. José de Almeida
| | - Inês Silva
- Rheumatology, Hospital de Egas Moniz, Lisbon, Portugal
| | - Manuela Costa
- Rheumatology, Hospital de Egas Moniz, Lisbon, Portugal
| | | | | |
Collapse
|
|
12
|
Zhu J, Lai J, Liu X, Zhao X, Tao R, Kang M, Huang X, Wang L, Wu F, Pan X, Su G. Clinical characteristics and prognosis of childhood-onset lupus mesenteric vasculitis as the initial presentation-a case-control study. Arthritis Res Ther 2023; 25:248. [PMID: 38124151 PMCID: PMC10731905 DOI: 10.1186/s13075-023-03237-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Accepted: 12/13/2023] [Indexed: 12/23/2023] Open
Abstract
BACKGROUND Lupus mesenteric vasculitis (LMV) as initial presentation is rare, especially in childhood-onset systemic lupus erythematosus (cSLE). It is a critical complication of lupus. At present, the research on cSLE with LMV as the initial presentation is few. The aim of this study was to analyze the clinical characteristics and prognosis of cSLE with LMV in the Chinese population, compared with non-LMV cSLE. METHODS A retrospective case-controlled study was conducted on 55 cSLE patients between July 2018 and July 2021. The clinical data, laboratory findings, imaging, treatment, and follow-up data were collected and compared between the two groups of cSLE with LMV and non-LMV. Non-LMV cSLE patients were matched according to the age and sex of LMV patients. RESULTS A total of 11 cSLE patients with LMV as the LMV group and 44 cSLE patients without LMV as the non-LMV group were included. The average age of onset was 12.55 ± 1.57 years old, the male-to-female ratio was 2:9, and high disease activity was observed in the LMV group. Abdominal pain was most common in LMV. Compared with the non-LMV, the percentage of abdominal pain, vomiting, abdominal distension, and diarrhea was higher, and gastrointestinal tract, serous cavity, kidney, and lung damage were higher in the LMV group (P < 0.05). In abdominal-enhanced CT, the percentage of intestinal wall thickening, peritoneal effusion, mesenteric vascular enhancement, hydronephrosis with ureteral dilatation, intestinal congestion, and gastric mucosa thickening in the LMV group were higher than those in the non-LMV group (P < 0.05). The percentage of receiving methylprednisolone pulse combined with cyclophosphamide pulse therapy in LMV was higher than in non-LMV. The clinical symptoms disappeared quickly, and there were no deaths in the LMV group. Compared with the non-LMV group, the 24-h urinary protein was higher, the complement C3 was lower, and the disease activity was higher in the LMV group (P < 0.05). CONCLUSIONS LMV often occurs in 12 ~ 13-year-old girls with high disease activity of cSLE. Abdominal pain is the most common and more susceptible to damage to the kidney, serous cavity, and lung in cSLE with LMV. Methylprednisolone pulse combined with CTX pulse therapy is effective. After the treatment above, cSLE with LMV has a good prognosis, but the overall recovery is worse than non-LMV patients.
Collapse
Affiliation(s)
- Jia Zhu
- Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Jianming Lai
- Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Xiaohui Liu
- Department of Rheumatology and Immunology, Jiangxi Provincial Children's Hospital, No. 1666, Diezihu Avenue, Honggutan District, Nanchang, 330013, Jiangxi Province, China
| | - Xue Zhao
- Department of Pediatric, The Second Hospital of Hebei Medical University, No. 215, Heping West Road, Xinhua District, Shijiazhuang, 050000, Hebei Province, China
| | - Ran Tao
- Department of Radiology, Children's Hospital, Capital Institute of Pediatrics, NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Min Kang
- Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Xiaolan Huang
- Experimental Research Center, Capital Institute of Pediatrics, NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Li Wang
- Capital Institute of Pediatrics, NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Fengqi Wu
- Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China
| | - Xiaoping Pan
- Department of Information Management, The National Center for Women's and Children's Health of the Chinese Center for Disease Control and Prevention, NO.27 Nanwei Road, Xicheng District, Beijing, 100050, China.
| | - Gaixiu Su
- Department of Rheumatology and Immunology, Children's Hospital, Capital Institute of Pediatrics. , NO.2 Yabao Road, Chaoyang District, Beijing, 100020, China.
| |
Collapse
|
|
13
|
Sakuma S, Abe Y, Takeuchi M, Makiyama A, Tamura N. 18 F-FDG PET/CT Reveals Localized Inflammation in Lupus Enteritis. Clin Nucl Med 2023; 48:890-893. [PMID: 37486305 DOI: 10.1097/rlu.0000000000004783] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/25/2023]
Abstract
ABSTRACT Lupus enteritis is classified into the colon poly-ulcerative type and the small intestine ischemic serositis type. Colon poly-ulcerative lupus enteritis is a disease that is mainly due to mesenteric arteritis. In recent years, 18 F-FDG PET/CT has been frequently used to assess the extent of the disease in patients with systemic vasculitis. We present the case report of 18 F-FDG PET/CT results in a 57-year-old woman with colon poly-ulcerative lupus enteritis.
Collapse
Affiliation(s)
- Shota Sakuma
- From the Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan
| | | | | | | | | |
Collapse
|
|
14
|
Huang H, Li P, Zhang D, Zhang MX, Yu K. Acute flare of systemic lupus erythematosus with extensive gastrointestinal involvement: A case report and review of literature. World J Gastrointest Surg 2023; 15:2074-2082. [PMID: 37901723 PMCID: PMC10600777 DOI: 10.4240/wjgs.v15.i9.2074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2023] [Revised: 07/09/2023] [Accepted: 07/28/2023] [Indexed: 09/21/2023] Open
Abstract
BACKGROUND Lupus mesenteric vasculitis (LMV) is a serious condition that may occur as an acute manifestation of gastrointestinal (GI) involvement and is not easily diagnosed by physicians. Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening. CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water. Laboratory investigations, physical examinations, and enhanced abdominal computed tomography (CT) suggested systemic lupus erythematosus complicated by LMV. She received treatments, such as GI decompression, somatostatin, glucocorticoids, and immunosuppressants, and was evaluated using color ultrasonography. Twenty days later, the patient reported no stomach discomfort and was able to consume semi-liquid food. Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly. One year after discharged, she recovered with methylprednisolone being tapered to 4 mg per day, mycophenolate mofetil to 0.75 g bid, and hydroxychloroquine to 0.2 g bid; however, only C3 complement level was slightly below the normal level. CONCLUSION Early diagnosis of LMV is essential for successful treatment; this depends on a combination of clinical manifestations, laboratory investigations, and imaging findings. Enhanced CT is preferred, but ultrasonography can be used for prompt screening and follow-up.
Collapse
Affiliation(s)
- Hua Huang
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Ping Li
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Dan Zhang
- Department of Nutrition, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Ming-Xuan Zhang
- Department of Rheumatology and Immunology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| | - Kai Yu
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang 110000, Liaoning Province, China
| |
Collapse
|
|
15
|
Chen YL, Meng J, Li C. Intestinal perforation with systemic lupus erythematosus: A systematic review. Medicine (Baltimore) 2023; 102:e34415. [PMID: 37543816 PMCID: PMC10402941 DOI: 10.1097/md.0000000000034415] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2023] [Revised: 06/27/2023] [Accepted: 06/29/2023] [Indexed: 08/07/2023] Open
Abstract
Intestinal perforation (IP) is a rare complication of systemic lupus erythematosus (SLE), and the timely diagnosis and treatment of IP are necessary to prevent death. In this study, the clinical features of IP in SLE were described in an attempt to enhance its understanding to reduce mortality. The clinical data of IP in SLE from 1984 to 2022 were retrospectively collected. A total of 18 patients were enrolled, and data on clinical symptoms, preoperative evaluation, surgical procedures, and postoperative outcomes were collected and retrospectively analyzed. The analysis included 15 females and 3 males, with a mean age of 49.2 years. Fifteen patients (83.3%) had a history of the disease for >5 years, and the SLE disease activity index score of 1 (5.6%) patient was <5 points and that of 17 (94.4%) patients was >10 points. A total of 9 (50%), 5 (27.7%), 3 (16.7%), and 1 (5.6%) patient had lesions in the rectum, colon, ileum, and both ileum and appendix, respectively. The cause of perforation in 12 (66.7%) patients was lupus mesenteric vasculitis and in 3 (16.7%) patients was chronic inflammation. Seven (38.9%) patients had other immune system diseases. All patients were treated with steroids and surgical treatment. However, 5 patients died after surgery. A disease duration of >5 years, SLE disease activity index score of >10, nonstandard use of steroids, and concomitant presence of other immune system diseases are the possible risk factors of IP in SLE. The most common site of perforation was the rectum, which was caused by lupus mesenteric vasculitis. The results suggest that the key to successfully manage such cases is early diagnosis, aggressive resuscitation, antibiotics, steroid therapy, and prompt surgical intervention.
Collapse
Affiliation(s)
- Ya Lan Chen
- Department of Gastroenterology, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| | - Jie Meng
- Department of Gastroenterology, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| | - Cong Li
- Department of Hepatobiliary Surgery, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| |
Collapse
|
|
16
|
Bradley IC, Trivedi B, Brockman MJ, Hassan M, Sotelo J, Okopie T, Dihowm F. Superior Mesenteric Artery Syndrome in Systemic Lupus Erythematosus. Cureus 2023; 15:e42032. [PMID: 37593277 PMCID: PMC10431686 DOI: 10.7759/cureus.42032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/17/2023] [Indexed: 08/19/2023] Open
Abstract
Although the gastrointestinal (GI) manifestations of systemic lupus erythematosus (SLE) are relatively less reported, they are common and occur in approximately half of individuals with SLE. These symptoms vary and include, but are not limited to, oral ulceration, dysphagia, nausea, vomiting, diarrhea, abdominal pain, and intestinal perforation. Gastrointestinal manifestations are often triggered by an inciting event, such as an infection or the side effects of medication. This case report presents a rare GI-related SLE complication, namely superior mesenteric artery syndrome.
Collapse
Affiliation(s)
- India C Bradley
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Bhavi Trivedi
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Michael J Brockman
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Mariam Hassan
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Jose Sotelo
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Tobi Okopie
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| | - Fatma Dihowm
- Internal Medicine, Texas Tech University Health Sciences Center El Paso, El Paso, USA
| |
Collapse
|
|
17
|
Paramaiswari A, Kusumawati A, Nugroho DB. Severe Abdominal Pain and Multi-Organ Involvement in a Young Woman With Systemic Lupus Erythematosus. J Med Cases 2023; 14:196-199. [PMID: 37435108 PMCID: PMC10332872 DOI: 10.14740/jmc4065] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/22/2023] [Accepted: 05/24/2023] [Indexed: 07/13/2023] Open
Abstract
Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by diverse clinical manifestations and a broad spectrum of disease course and prognosis. Often presenting over an extended period, delays in diagnosis can significantly influence patient management and survival, particularly when faced with rare complications such as digestive system manifestations. This case report uniquely highlights the diagnostic and therapeutic challenges posed by severe abdominal pain in a young woman suspected of SLE, with a symptom often masked by steroid therapy or immunosuppression. The diagnostic journey, which led to the identification of SLE as the cause of abdominal pain, involved differentiating SLE from various abdominal pathologies including abdominal vasculitis, gastrointestinal syndrome, antiphospholipid antibody syndrome, pancreatitis, urinary tract infections, and obstetric-gynecological abnormalities. This case underlines the critical need for accurate, timely diagnosis, and targeted therapy in managing SLE, emphasizing the potential implications of such complexities on patient outcomes.
Collapse
Affiliation(s)
- Ayu Paramaiswari
- Department of Internal Medicine, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta 55281, Indonesia
| | - Anita Kusumawati
- Department of Internal Medicine, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta 55281, Indonesia
| | - Dhite Bayu Nugroho
- Department of Internal Medicine, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta 55281, Indonesia
| |
Collapse
|
|
18
|
Muñoz-Urbano M, Sanchez-Bautista J, Ramírez A, Santamaría-Alza Y, Quintero-González DC, Vanegas-García AL, Vásquez G, González LA. Lupus enteritis: A 10-year experience in a single Latin American center. Lupus 2023:9612033231175782. [PMID: 37184366 DOI: 10.1177/09612033231175782] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/16/2023]
Abstract
OBJECTIVE The objective is to compare the clinical and laboratory characteristics of systemic lupus erythematosus (SLE) patients with and without lupus enteritis (LE) and to identify the factors associated with the occurrence of LE. METHODS We performed a retrospective, case-control study in hospitalized patients with SLE who were admitted to our tertiary hospital between January 2012 and December 2021. Sixteen LE patients (cases) were matched (1:3 ratio) for sex and birth year with 48 non-LE patients (controls). Univariable and multivariable logistic regression analyses were used to identify the variables associated with LE. RESULTS Of 2,479 SLE patients who were admitted to our hospital as inpatients, 16 (0.65%) were diagnosed as having LE. All patients, cases and controls, were of Mestizo ethnicity. SLE was diagnosed simultaneously with the first episode of LE in 10 (62.5%) patients. The median time from SLE diagnosis to the first episode of LE was 7 (IQR 0-78) months. LE patients had a shorter median disease duration [7 (0-78) vs 34 (9.5-79) months], and a significantly longer hospital stay (28.3 ± 15.8 vs 6.5 ± 7.9 days, p < 0.001) than non-LE patients. Most LE patients (93.8%) had concomitant lupus nephritis. LE patients had higher SLEDAI-2K scores than those without LE (20.5 ± 9.4 vs 9.8 ± 10.4, p < 0.001). By multivariable analysis, a higher SLEDAI-2K score (OR 1.10, 95% CI 1.02-1.18; p = 0.015) was independently associated with LE occurrence after adjusting for cutaneous involvement, lymphocyte count, serum creatinine, and serum complement C4. Recurrence was observed in two patients (12.5%), both with a bowel wall thickening > 8 mm. The two patients with large intestine-dominant LE developed intestinal pseudo-obstruction. No patient had life-threatening complications (intestinal hemorrhage, infarction, or perforation), and there were no deaths induced directly by LE itself. CONCLUSION In patients of Mestizo ethnicity, LE occurs during the early course of SLE, frequently is one of the presenting manifestations of SLE, and in most cases, it presents with concomitant lupus nephritis. Higher levels of disease activity at diagnosis were independently associated with LE occurrence and when recurrences occur, they do so in the context of severe wall thickness.
Collapse
Affiliation(s)
- Marcela Muñoz-Urbano
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Julián Sanchez-Bautista
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Andrés Ramírez
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Yeison Santamaría-Alza
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Diana C Quintero-González
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Adriana-Lucía Vanegas-García
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Gloria Vásquez
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Luis A González
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| |
Collapse
|
|
19
|
Xu H, Meng X, Wang L, Zhu G, Fan X, Li R, Fei Y, Yang H, Zhao L. Dietary patterns and life-styles of patients with gastrointestinal involvement of systemic lupus erythematosus: Questionnaire survey from a tertiary center of China. Lupus 2023; 32:477-488. [PMID: 36749733 DOI: 10.1177/09612033231156075] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
OBJECTIVE To investigate the dietary patterns and lifestyles of patients with lupus gastrointestinal (GI) involvement and to reveal the possible role of organ-specific involvement of systemic lupus erythematosus (SLE) on daily diet. METHODS Patients with SLE complicated with gastrointestinal involvement (SLE-GI) admitted to Peking Union Medical College Hospital (PUMCH) from January 2010 to September 2021 were enrolled. Age- and sex-matched SLE patients with lupus nephritis (SLE-LN) but free of other internal organs involvement who were admitted during the same period were enrolled as disease controls at the ratio of 1:1. In addition, a group of age- and sex-matched healthy people were also included as healthy controls (HCs). Questionnaires were distributed to these patients and HC to collect their dietary patterns and lifestyle information. Clinical features, dietary and lifestyle habits were compared between the two groups of patients and HC. RESULTS The questionnaire survey showed that compared with HC, the SLE-GI group had higher proportions of vegetarians (p = 0.014) and a lower proportion of omnivores (p = 0.058). A higher percentage of SLE-GI patients reported a traditional Chinese medicine (p = 0.018) taken history and surgical history (p = 0.014). They also less likely to take fried/pickled food (p = 0.042) and dietary supplements (p = 0.024) than HC. Higher percentages of SLE-GI patients and SLE-LN patients preferred self-catering (87.5% and 94.3%) over take-out food than HC (70.8%) (p = 0.127 and p = 0.016). No significant difference on drinking preference among the three groups, but it seemed more SLE-GI patients consumed yogurt than HC (p = 0.097). The SLE-LN patients were also found to have lower frequencies of staying up late (p = 0.005). The SLE-GI group also presented higher positivity rates for anti-SSA (69.6% vs. 45.7%, p = 0.020) and anti-SSB antibodies (32.6% vs. 10.9%, p = 0.011) but lower positivity rates for anti-dsDNA antibodies (30.4% vs. 82.6%, p < 0.001) compared with the SLE-LN group. CONCLUSION The dietary patterns, life-styles and autoantibody spectrum of SLE-GI patients differed greatly from those of SLE-LN patients and healthy people. These factors may reflect the influence of disease and organ involvement modes on patients' daily life and may contribute partly to the systemic involvement in SLE.
Collapse
Affiliation(s)
- Haojie Xu
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Xia Meng
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Lu Wang
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Gaoqi Zhu
- Department of Rheumatology and Immunology, 74539The First Affiliated Hospital of Bengbu Medical College, Bengbu, China
| | - Xiaoyun Fan
- Department of Rheumatology and Immunology, 74539The First Affiliated Hospital of Bengbu Medical College, Bengbu, China
| | - Rongli Li
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Yunyun Fei
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Huaxia Yang
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Lidan Zhao
- Department of Rheumatology and Clinical Immunology, 34732Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.,National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| |
Collapse
|
|
20
|
Aso K, Kono M, Abe N, Fujieda Y, Kato M, Atsumi T. Anti-ganglionic nicotinic acetylcholine receptor α3 subunit antibody as a potential biomarker associated with lupus enteritis. Mod Rheumatol 2023; 33:154-159. [PMID: 35107135 DOI: 10.1093/mr/roac006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2021] [Revised: 12/20/2021] [Accepted: 12/25/2021] [Indexed: 01/05/2023]
Abstract
OBJECTIVES We aimed to identify the clinical significance of anti-ganglionic nicotinic acetylcholine receptor α3 subunit (gAChRα3) antibodies (Abs) in patients with systemic lupus erythematosus (SLE). METHODS This retrospective study comprised adult patients with SLE who visited our hospital from 2006 through 2019. Anti-gAChRα3 Abs were measured in the sera of patients with SLE using a luciferase immunoprecipitation system assay. The clinical features of the patients with or without anti-gAChRα3 Abs were compared. We evaluated whether the Abs predict a specific manifestation and affect its development or relapse rate. RESULTS Among 144 patients, anti-gAChRα3 Abs were detected in 29 patients. Lupus enteritis (LE) was more frequently seen in anti-gAChRα3 Ab-positive patients than negative patients. The levels of anti-gAChRα3 Abs were significantly higher in patients with LE than those with other lupus manifestations. Logistic regression analysis revealed the anti-gAChRα3 Abs were independent predictors for LE (odds ratio 6.2, 95% confidence interval 1.9-20.3, p = .002). Kaplan-Meier analysis showed the rate of LE development or relapse from the time of sera collection was higher in anti-gAChRα3 Ab-positive patients than in negative patients (p < .001). CONCLUSION Anti-gAChRα3 Abs could be a predictive biomarker for the development or relapse of LE.
Collapse
Affiliation(s)
- Kuniyuki Aso
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| | - Michihito Kono
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| | - Nobuya Abe
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| | - Yuichiro Fujieda
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| | - Masaru Kato
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| | - Tatsuya Atsumi
- Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaidô, Japan
| |
Collapse
|
|
21
|
Milne M, Sims C, Anderson DR, Johannemann A, Leverenz D, Criscione-Schreiber L, Ardalan K. A Rare Manifestation of a Rare Disease: The Importance of Thinking Outside the Box in a Patient With Complex Dermatomyositis. Arthritis Care Res (Hoboken) 2022; 74:1943-1949. [PMID: 35507472 DOI: 10.1002/acr.24905] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2021] [Revised: 03/03/2022] [Accepted: 04/28/2022] [Indexed: 02/05/2023]
Affiliation(s)
- Megan Milne
- Duke University School of Medicine, Durham, North Carolina
| | - Catherine Sims
- Duke University School of Medicine, Durham, North Carolina
| | | | | | - David Leverenz
- Duke University School of Medicine, Durham, North Carolina
| | | | - Kaveh Ardalan
- Duke University School of Medicine, Durham, North Carolina
| |
Collapse
|
|
22
|
Alsolaimani R. Mesenteric Vasculitis and Urinary System Involvement Presenting As the Initial Manifestations of Systemic Lupus Erythematosus Treated Successfully With Glucocorticoids and Rituximab: A Case Report. Cureus 2022; 14:e31474. [DOI: 10.7759/cureus.31474] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/14/2022] [Indexed: 11/16/2022] Open
|
|
23
|
Seitz L, Seitz P, Pop R, Lötscher F. Spectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases. Curr Rheumatol Rep 2022; 24:352-370. [PMID: 36166150 PMCID: PMC9513304 DOI: 10.1007/s11926-022-01086-2] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/26/2022] [Indexed: 11/24/2022]
Abstract
PURPOSE OF REVIEW To provide a comprehensive overview of the spectrum of large and medium vessel vasculitis in adults with primary vasculitides, arthritides, connective tissue, and fibroinflammatory diseases as well as vasculitis mimics, for an efficient differential diagnosis and initial diagnostic approach. RECENT FINDINGS Imaging has had a tremendous impact on the diagnosis of medium to large vessel vasculitis, now often replacing histopathologic confirmation and identifying new disease manifestations (e.g., intracranial disease in giant cell arteritis; vascular manifestations of IgG4-related disease). Novel diseases or syndromes involving blood vessels have been described (e.g., VEXAS-Syndrome with polychondritis). The use of the terms "medium" or "large" vessel varies considerably between medical specialties. The differential diagnosis of large and medium vessel vasculitis is becoming increasingly complex as new entities or disease manifestations of known inflammatory rheumatic diseases are regularly identified. A more precise and widely recognized definition of the vessel sizes would make future research more comparable.
Collapse
Affiliation(s)
- Luca Seitz
- Department of Rheumatology and Immunology, Inselspital, University Hospital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland.
- Immunodeficiency Laboratory, Department of Biomedicine, University Hospital and University of Basel, Basel, Switzerland.
| | - Pascal Seitz
- Department of Rheumatology and Immunology, Inselspital, University Hospital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland
| | - Roxana Pop
- Department of Infectious Diseases and Hospital Hygiene, University Hospital, University of Zurich, Zurich, Switzerland
| | - Fabian Lötscher
- Department of Rheumatology and Immunology, Inselspital, University Hospital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland
| |
Collapse
|
|
24
|
Zargar P, Gandhi S, Kotwal VS. Target Sign and Abdominal Pain: An Approach to Diagnosis and Treatment. Gastroenterology 2022; 163:1183-1185. [PMID: 35667406 DOI: 10.1053/j.gastro.2022.05.053] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2022] [Accepted: 05/26/2022] [Indexed: 12/02/2022]
Affiliation(s)
- Pejman Zargar
- Division of Gastroenterology, John H. Stroger, Jr Hospital of Cook County, Chicago, Illinois.
| | - Seema Gandhi
- Division of Gastroenterology, John H. Stroger, Jr Hospital of Cook County, Chicago, Illinois
| | - Vikram S Kotwal
- Division of Gastroenterology, John H. Stroger, Jr Hospital of Cook County, Chicago, Illinois
| |
Collapse
|
|
25
|
Huang SC, Chan YL, Cheng HT, Goh ZNL, Wong YC, Seak CK, Seak JCY, Li CH, Chen HY, Seak CJ. When a Rapid Accurate Diagnosis Changes Therapeutic Approach: Recognizing Acute Abdominal Pain with Ascites as a Possible Presentation of Systemic Lupus Erythematosus. Diagnostics (Basel) 2022; 12:2605. [PMID: 36359449 PMCID: PMC9689037 DOI: 10.3390/diagnostics12112605] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2022] [Revised: 10/03/2022] [Accepted: 10/24/2022] [Indexed: 08/30/2023] Open
Abstract
Systemic lupus erythematosus (SLE) is a chronic, multi-organ autoimmune disease which rarely presents with peritoneal involvement. As such, its diagnosis in the emergency department (ED) based on a clinical presentation of gastrointestinal symptoms is extremely challenging. Yet, reaching such a diagnosis in the ED is crucial for avoiding unnecessary surgical intervention and initiating early glucocorticoid therapy to maximise patient outcomes. Here, we report a case of newly diagnosed SLE in a 28-year-old lady who presented atypically and unusually with abdominal pain and ascites. She required extensive but methodical investigations, and was eventually diagnosed with lupus mesenteric vasculitis with underlying newly diagnosed SLE in the ED. The patient was promptly treated with methylprednisolone resulting in marked clinical improvement. Emergency physicians should be mindful of abdominal pain with ascites as an extremely rare but important clinical presentation of SLE. Early diagnosis and commencement of glucocorticoid therapy in these patients are crucial in halting disease progression and averting the need for surgical intervention.
Collapse
Affiliation(s)
- Szu-Cheng Huang
- Department of Emergency Medicine, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
| | - Yi-Ling Chan
- Department of Emergency Medicine, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
| | - Hao-Tsai Cheng
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
- Department of Gastroenterology and Hepatology, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
- Department of Gastroenterology and Hepatology, New Taipei Municipal Tucheng Hospital, New Taipei City 23652, Taiwan
| | | | - Yon-Cheong Wong
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
- Department of Medical Imaging and Intervention, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
| | - Chen-Ken Seak
- Sarawak General Hospital, Kuching 93586, Sarawak, Malaysia
| | | | - Chih-Huang Li
- Department of Emergency Medicine, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
| | - Hsien-Yi Chen
- Department of Emergency Medicine, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
| | - Chen-June Seak
- Department of Emergency Medicine, Lin-Kou Medical Center, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan
- College of Medicine, Chang Gung University, Taoyuan 33302, Taiwan
- Department of Emergency Medicine, New Taipei Municipal Tucheng Hospital, New Taipei City 23652, Taiwan
| |
Collapse
|
|
26
|
Alharbi S. Gastrointestinal Manifestations in Patients with Systemic Lupus Erythematosus. Open Access Rheumatol 2022; 14:243-253. [PMID: 36281321 PMCID: PMC9587305 DOI: 10.2147/oarrr.s384256] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2022] [Accepted: 10/11/2022] [Indexed: 11/05/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disorder of unknown etiology. Women of childbearing age are affected approximately nine times more often than men. Its presentation and course are highly variable, ranging from mild to fulminant systemic disease. Any organ can be affected by SLE. Although less common than in other systems, such as the skin, joints, and kidneys, 40%–60% of SLE patients have gastrointestinal (GI) involvement. SLE can affect any part of the GI tract, from the mouth to the anus. GI manifestations can be caused by SLE, medication-related side effects, or non-SLE causes including infection. This article reviews the most common types of GI involvement associated with SLE.
Collapse
Affiliation(s)
- Samar Alharbi
- Department of Medicine, College of Medicine, Taibah University, Medina, Saudi Arabia,Correspondence: Samar Alharbi, Department of Medicine, College of Medicine, Taibah University, Medina, 42312-3779, Saudi Arabia, Tel +96 6553018777, Fax +96 648461172, Email
| |
Collapse
|
|
27
|
Amouei M, Momtazmanesh S, Kavosi H, Davarpanah AH, Shirkhoda A, Radmard AR. Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Insights Imaging 2022; 13:143. [PMID: 36057741 PMCID: PMC9440973 DOI: 10.1186/s13244-022-01284-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Accepted: 08/04/2022] [Indexed: 11/21/2022] Open
Abstract
Diagnosis of intestinal vasculitis is often challenging due to the non-specific clinical and imaging findings. Vasculitides with gastrointestinal (GI) manifestations are rare, but their diagnosis holds immense significance as late or missed recognition can result in high mortality rates. Given the resemblance of radiologic findings with some other entities, GI vasculitis is often overlooked on small bowel studies done using computed tomography/magnetic resonance enterography (CTE/MRE). Hereon, we reviewed radiologic findings of vasculitis with gastrointestinal involvement on CTE and MRE. The variety of findings on MRE/CTE depend upon the size of the involved vessels. Signs of intestinal ischemia, e.g., mural thickening, submucosal edema, mural hyperenhancement, and restricted diffusion on diffusion-weighted imaging, are common in intestinal vasculitis. Involvement of the abdominal aorta and the major visceral arteries is presented as concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels. Such findings can be observed particularly in large- and medium-vessel vasculitis. The presence of extra-intestinal findings, including within the liver, kidneys, or spleen in the form of focal areas of infarction or heterogeneous enhancement due to microvascular involvement, can be another radiologic clue in diagnosis of vasculitis. The link between the clinical/laboratory findings and MRE/CTE abnormalities needs to be corresponded when it comes to the diagnosis of intestinal vasculitis.
Collapse
Affiliation(s)
- Mehrnam Amouei
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
| | - Sara Momtazmanesh
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
| | - Hoda Kavosi
- Department of Rheumatology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Amir H. Davarpanah
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, USA
| | - Ali Shirkhoda
- Department of Radiological Science, University of California at Irvine, Irvine, USA
| | - Amir Reza Radmard
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, North Kargar St., Tehran, 14117 Iran
| |
Collapse
|
|
28
|
Zhang W, Huang G, Lin J, Lin Q, Zheng K, Hu S, Zheng S, Du G, Matucci-Cerinic M, Furst DE, Wang Y. Predictive model of risk and severity of enteritis in systemic lupus erythematosus. Lupus 2022; 31:1226-1236. [PMID: 35750508 DOI: 10.1177/09612033221110743] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
INTRODUCTION To describe the clinical and laboratory features of systemic lupus erythematosus (SLE) enteritis and to establish a predictive model of risk and severity of lupus enteritis (LE). METHODS Records of patients with SLE complaining about acute digestive symptoms were reviewed. The predictive nomogram for the diagnosis of LE was constructed by using R. The accuracy of the model was tested with correction curves. The receiver operating characteristic curve (ROC curve) program and a Decision curve analysis (DCA) were used for the verification of LE model. Receiver operating characteristic curve was also employed for evaluation of factors in the prediction of severity of LE. RESULTS During the eight year period, 46 patients were in the LE group, while 32 were in the non-LE group. Abdominal pain, emesis, D-dimer >5 μg/mL, hypo-C3, and anti-SSA positive remained statistically significant and were included into the prediction model. Area under the curve (AUC) of ROC curve in this model was 0.909. Correction curve indicated consistency between the predicted rate and actual diagnostic rates. The DCA showed that the LE model was of benefit. Forty-four patients were included in developing the prediction model of LE severity. Infection, SLE disease activity index (SLEDAI), CT score, and new CT score were validated as risk factors for LE severity. The AUC of the combined SLEDAI, infection and new CT score were 0.870. CONCLUSION The LE model exhibits good predictive ability to assess LE risk in SLE patients with acute digestive symptoms. The combination of SLEDAI, infection, and new CT score could improve the assessment of LE severity.
Collapse
Affiliation(s)
- Weijin Zhang
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Guohai Huang
- Department of Blood Purification, 499791Shantou Central Hospital, Shantou, China
| | - Jianqun Lin
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Qisheng Lin
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Kedi Zheng
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Shijian Hu
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Shaoyu Zheng
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China
| | - Guangzhou Du
- Department of Radiology, 499791Shantou Central Hospital, Shantou, China
| | - Marco Matucci-Cerinic
- Department of Experimental and Clinical Medicine, Division of Rheumatology, Careggi University Hospital, 9300University of Florence, Florence, Italy.,Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy
| | - Daniel E Furst
- Department of Experimental and Clinical Medicine, Division of Rheumatology, Careggi University Hospital, 9300University of Florence, Florence, Italy.,Division of Rheumatology, Department of Medicine, 8783University of California at Los Angeles, USA.,University of Washington, Seattle, WA, USA
| | - Yukai Wang
- Department of Rheumatology and Immunology, 499791Shantou Central Hospital, Shantou, China.,Department of Experimental and Clinical Medicine, Division of Rheumatology, Careggi University Hospital, 9300University of Florence, Florence, Italy
| |
Collapse
|
|
29
|
Yu SJ, Heo JH, Choi EJ, Kim JH, Lee HS, Kim SY, Lim JH. Role of multidetector computed tomography in patients with acute infectious colitis. World J Clin Cases 2022; 10:3686-3697. [PMID: 35647171 PMCID: PMC9100710 DOI: 10.12998/wjcc.v10.i12.3686] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2021] [Revised: 09/14/2021] [Accepted: 03/06/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The role of multidetector computed tomography (MDCT) in patients with acute infectious colitis is still unclear.
AIM To examine the usefulness of MDCT in distinguishing the etiology of acute infectious colitis.
METHODS Overall, 244 patients who met the criteria for acute infectious colitis and visited the Hospital from February 2015 to 2018 were retrospectively enrolled and divided into two groups (bacterial: 204, viral: 40) according to causes of acute colitis, based on stool PCR. Eleven MDCT parameters, including wall thickening, submucosal edema, mucosal enhancement, serosa involvement, empty colon sign, small bowel involvement, comb sign, continuous distribution, accordion sign, mucosal thickening, and lymph node enlargement, were constructed in a blinded fashion.
RESULTS MDCT parameters of wall thickening (OR: 13.60; 95%CI: 5.80–31.88; P < 0.001), submucosal edema (OR: 36.08; 95%CI: 13.54–96.13; P < 0.001), mucosal enhancement (OR: 22.55; 95%CI: 9.28–54.81; P < 0.001), serosal involvement (OR: 14.50; 95%CI: 3.33–63.23; P < 0.001), empty colon sign (OR: 6.68; 95%CI: 2.44–18.32; P < 0.001), continuous distribution (OR: 24.09; 95%CI: 9.38–61.90; P < 0.001), accordion sign (OR: 9.02; 95%CI: 1.12–72.35; P = 0.038), mucosal thickening (OR: 46.41; 95%CI: 10.38–207.51; P < 0.001), and lymph node enlargement (OR: 4.39; 95%CI: 1.22–15.72; P = 0.023) were significantly associated with bacterial colitis. At least one positive finding in four CT outcomes (submucosal edema, mucosal enhancement, continuous distribution, mucosal thickening) in summer showed a high probability of bacterial colitis (sensitivity, 41.67; specificity, 92.50; OR: 24.95).
CONCLUSION MDCT provides many clues that can be useful in suggesting a specific etiology of acute infectious colitis.
Collapse
Affiliation(s)
- Seung Jung Yu
- Department of Internal Medicine, Inje University Busan Paik Hospital, Busan 47392, South Korea
| | - Jae Hyuk Heo
- Department of Internal Medicine, Inje University Busan Paik Hospital, Busan 47392, South Korea
| | - Eun Jeong Choi
- Department of Internal Medicine, Inje University Busan Paik Hospital, Busan 47392, South Korea
| | - Jong Hyuk Kim
- Department of Radiology, Myongji Hospital, Hanyang University College of Medicine, Goyang 10475, South Korea
| | - Hong Sub Lee
- Department of Internal Medicine, Inje University Busan Paik Hospital, Busan 47392, South Korea
| | - Sun Young Kim
- Department of Cancer Control and Population Health, National Cancer Center, Goyang 10408, South Korea
| | - Jae Hoon Lim
- Department of Radiology, Myongji Hospital, Hanyang University College of Medicine, Goyang 10475, South Korea
| |
Collapse
|
|
30
|
Yamaguchi T. Characteristic target sign of the small intestine in a case of lupus enteritis. J Gen Fam Med 2021; 23:187-188. [PMID: 35509338 PMCID: PMC9062572 DOI: 10.1002/jgf2.517] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2021] [Revised: 11/27/2021] [Accepted: 12/20/2021] [Indexed: 11/18/2022] Open
Affiliation(s)
- Toshimasa Yamaguchi
- Primary Care and Advanced Triage Section Osaka City General Hospital Osaka Japan
| |
Collapse
|
|
31
|
Leone P, Prete M, Malerba E, Bray A, Susca N, Ingravallo G, Racanelli V. Lupus Vasculitis: An Overview. Biomedicines 2021; 9:1626. [PMID: 34829857 PMCID: PMC8615745 DOI: 10.3390/biomedicines9111626] [Citation(s) in RCA: 38] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2021] [Revised: 10/29/2021] [Accepted: 11/03/2021] [Indexed: 12/21/2022] Open
Abstract
Lupus vasculitis (LV) is one of the secondary vasculitides occurring in the setting of systemic lupus erythematosus (SLE) in approximately 50% of patients. It is most commonly associated with small vessels, but medium-sized vessels can also be affected, whereas large vessel involvement is very rare. LV may involve different organ systems and present in a wide variety of clinical manifestations according to the size and site of the vessels involved. LV usually portends a poor prognosis, and a prompt diagnosis is fundamental for a good outcome. The spectrum of involvement ranges from a relatively mild disease affecting small vessels or a single organ to a multiorgan system disease with life-threatening manifestations, such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex. Treatment depends upon the organs involved and the severity of the vasculitis process. In this review, we provide an overview of the different forms of LV, describing their clinical impact and focusing on the available treatment strategies.
Collapse
Affiliation(s)
- Patrizia Leone
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Marcella Prete
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Eleonora Malerba
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Antonella Bray
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Nicola Susca
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Giuseppe Ingravallo
- Section of Pathology, Department of Emergency and Organ Transplantation, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy;
| | - Vito Racanelli
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| |
Collapse
|
|
32
|
Chen L, He Q, Luo M, Gou Y, Jiang D, Zheng X, Yan G, He F. Clinical features of lupus enteritis: a single-center retrospective study. Orphanet J Rare Dis 2021; 16:396. [PMID: 34565417 PMCID: PMC8474739 DOI: 10.1186/s13023-021-02044-4] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2021] [Accepted: 09/19/2021] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Lupus enteritis (LEn) is a rare complication of systemic lupus erythematosus (SLE). Timely diagnosis and treatment of LEn are necessary to prevent the most serious consequences - intestinal perforation, gastrointestinal bleeding, and death. We compared the clinical features of SLE patients with and without LEn. METHODS The clinical data of LEn inpatients at Suining Central Hospital from July 2012 to June 2020 were examined. These LEn patients were matched (1:2 ratio) with concurrently hospitalized SLE patients who did not have LEn. The two groups were compared using multivariate logistic regression. RESULTS We compared SLE inpatients with LEn (n = 43) and SLE inpatients without LEn (n = 86) at our institution. Multivariate logistic regression showed that ascites (odds ratio [OR]: 9.961, 95%CI: 2.215-44.802, P = 0.003), hydronephrosis (OR: 28.060, 95%CI: 2.303-341.962, P = 0.009), leukopenia (OR: 5.890, 95%CI: 1.813-19.135, P = 0.003), reduced complement C3 level (OR: 4.791, 95%CI: 1.605-14.300, P = 0.005), and elevated immunoglobin (Ig)A level (OR: 4.040, 95%CI: 1.307-12.487, P = 0.015) were independently associated with LEn. Within the LEn group, abdominal pain was the most common abdominal symptom (88.4%), and increased mesenteric fat attenuation (74.4%) and bowel wall thickening (58.1%) were the most common computed tomography (CT) findings. Most LEn patients (88.4%) required high-dose glucocorticoid therapy (≥ 80 mg methylprednisolone/day), and cyclophosphamide was the most commonly used immunosuppressant (62.8%). CONCLUSIONS Abdominal pain was the most common clinical symptom of LEn. Abdominal CT provides important information for detection and diagnosis of LEn. Ascites, hydronephrosis, leukopenia, hypocomplementemia (C3), and increased IgA were independently associated with LEn.
Collapse
Affiliation(s)
- Long Chen
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China.,Department of Scientific Research Management, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Qin He
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Man Luo
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Yuxiao Gou
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Dan Jiang
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Xiaoqin Zheng
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Gaowu Yan
- Department of Radiology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China
| | - Fang He
- Department of Rheumatology and Immunology, Suining Central Hospital, No.127, West Desheng Rd., Chuanshan District, Suining, Sichuan Province, China.
| |
Collapse
|
|
33
|
Ileal perforation as an initial manifestation of systemic lupus erythematosus: A case report. Int J Surg Case Rep 2021; 87:106409. [PMID: 34555679 PMCID: PMC8461371 DOI: 10.1016/j.ijscr.2021.106409] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2021] [Revised: 08/13/2021] [Accepted: 09/12/2021] [Indexed: 11/21/2022] Open
Abstract
Introduction and importance Lupus enteritis is uncommon in patients with SLE and usually presents with anorexia, vomiting, and abdominal pain. Intestinal perforation as an initial manifestation of SLE is rare and can have a grave prognosis if not timely diagnosed. Case history We report an unusual case of a 22-year-old regularly menstruating female who presented with features of perforation peritonitis as an initial manifestation of lupus enteritis. Intraoperatively, a gangrenous ileal segment with multiple perforations was present. Thus, with an intraoperative diagnosis of perforation peritonitis, a gangrenous segment of the small bowel was resected and a double-barrel jejuno-ileostomy was created. Discussion Lupus enteritis manifesting initially as bowel perforation can be an uncommon cause of acute abdomen. A plain chest X-ray can show gas under the diaphragm suggesting bowel perforation. A contrast-enhanced CT scan of the abdomen is the gold standard in diagnosing lupus enteritis with a good prognosis on steroids. Conclusion Primary closure, resection, and anastomosis of small gut or diverting stoma are required for management of perforation. A high degree of clinical suspicion is required for early diagnosis thus preventing the grave prognosis of such an entity.
Bowel perforation in patients with SLE is rare. Diagnosis of this lupus enteritis is challenging owing to its rarity and non-specific presenting features. High degree of suspicion and timely diagnosis can prevent fatal complications.
Collapse
|
|
34
|
Frittoli RB, Vivaldo JF, Costallat LTL, Appenzeller S. Gastrointestinal involvement in systemic lupus erythematosus: A systematic review. J Transl Autoimmun 2021; 4:100106. [PMID: 34179742 PMCID: PMC8214088 DOI: 10.1016/j.jtauto.2021.100106] [Citation(s) in RCA: 32] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2021] [Accepted: 05/21/2021] [Indexed: 12/11/2022] Open
Abstract
INTRODUCTION Gastrointestinal involvement is a common complain observed in 40-60% of systemic lupus erythematosus (SLE) patients. We performed a systematic review of clinically severe and potential life-threatening gastrointestinal manifestations and discuss clinical presentation, pathogenesis and treatment. METHODS We performed a literature search in English literature using PubMed and Embase from 2000 to December 2020. The following MeSH terms: systemic lupus erythematosus, protein-losing enteropathy, ascites, pancreatitis, vasculitis, intestinal vasculitis, enteritis and diarrhea published in the English literature. RESULTS We identified 141 studies (case reports, case series and cohort studies). The most frequent presenting symptoms are acute abdominal pain, nausea, and vomiting. Many of the manifestations were associated with disease activity. Histological features are rarely available, but both vasculitis and thrombosis have been described. There is no treatment guideline. The majority of patients were treated with corticosteroids and the most common immunososupressant were azathioprine, cyclophosphamide and mycophenolate. CONCLUSION Vasculitis and thrombosis may be responsible for severe life-threatening manifestations such as pancreatitis, protein loosing gastroenteritis, acalculous cholecistyitis and enteritis.
Collapse
Affiliation(s)
- Renan Bazuco Frittoli
- Post-Graduation in Medical Pathophysiology, School of Medical Science, University of Campinas, Brazil
| | - Jéssica Fernandes Vivaldo
- Graduate Program in Child and Adolescent Health, School of Medical Science, University of Campinas, Brazil
| | - Lilian Tereza Lavras Costallat
- Department of Orthopedics, Rheumatology and Traumatology, School of Medical Science, University of Campinas, Campinas, São Paulo, 13083881, Brazil
| | - Simone Appenzeller
- Department of Medicine, School of Medical Science - State University of Campinas, Campinas, São Paulo, 13083881, Brazil
- Department of Orthopedics, Rheumatology and Traumatology, School of Medical Science, University of Campinas, Campinas, São Paulo, 13083881, Brazil
- Post-Graduation in Medical Pathophysiology, School of Medical Science, University of Campinas, Brazil
- Graduate Program in Child and Adolescent Health, School of Medical Science, University of Campinas, Brazil
| |
Collapse
|
|
35
|
Liu Z, Guo M, Cai Y, Zhao Y, Zeng F, Liu Y. A nomogram to predict the risk of lupus enteritis in systemic lupus erythematosus patients with gastroinctestinal involvement. EClinicalMedicine 2021; 36:100900. [PMID: 34041462 PMCID: PMC8144679 DOI: 10.1016/j.eclinm.2021.100900] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2021] [Revised: 04/26/2021] [Accepted: 04/26/2021] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Lupus enteritis (LE), a main cause of acute abdominal pain in systemic lupus erythematosus (SLE) patients, is a serious and potentially fatal complication. This study aimed to identify clinical serological indicators to establish a nomogram to assess LE in SLE patients with gastrointestinal manifestations. METHODS The clinical and laboratory data of SLE patients with gastrointestinal manifestations that were hospitalized in the West China Hospital from January 2010 to January 2020 were retrospectively analyzed. The least absolute shrinkage and selection operator logistic regression model was used to select potentially relevant features. Subsequently, a nomogram was developed using multivariable logistic analysis. The performance of the nomogram was evaluated using a receiver operating characteristic curve, a calibration curve, and decision curve analysis (DCA). FINDINGS We included a total of 8,505 SLE patients, of which 251 had experienced gastrointestinal manifestations. The patients were randomly divided into training (n = 176) and validation (n = 75) groups. The LRA (LE Risk Assessment) model consisted of 11 significantly associated variables, which included complement 4, antineutrophil cytoplasmic antibody, albumin, anion gap, age, d-dimer, platelet, serum chlorine, anti-Sjögren's-syndrome-related antigen A, anti-ribosomal P protein, and anti-ribonucleoprotein. In the training and validation cohorts, the areas under the curve were 0.919 (95% confidence interval [CI]: 0.876-0.962) and 0.870 (95% CI: 0.775-0.964), respectively. The nomogram demonstrated excellent performance in the calibration curve and DCA. INTERPRETATION The LRA model exhibits good predictive ability in assessing LE risk in SLE patients with gastrointestinal manifestations.
Collapse
Affiliation(s)
- Zhihui Liu
- Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China
| | - Min Guo
- Department of Rheumatology and Immunology, Chengdu Seventh People's Hospital, Chengdu, China
| | - Yurui Cai
- Department of Clinical Research Center, Dazhou Central Hospital, Dazhou, China
| | - Yi Zhao
- Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China
| | - Fanxin Zeng
- Department of Clinical Research Center, Dazhou Central Hospital, Dazhou, China
- Co-corresponding author at: Department of Clinical Research Center, Dazhou Central Hospital, Dazhou, China.
| | - Yi Liu
- Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China
- Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, China
- Institute of Immunology and Inflammation, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu, China
- Corresponding author at: Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China.
| |
Collapse
|
|
36
|
Wang H, Gao Q, Liao G, Ren S, You W. Clinico-Laboratory Features and Associated Factors of Lupus Mesenteric Vasculitis. Rheumatol Ther 2021; 8:1031-1042. [PMID: 34050908 PMCID: PMC8217476 DOI: 10.1007/s40744-021-00323-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2021] [Accepted: 05/13/2021] [Indexed: 11/18/2022] Open
Abstract
Introduction Lupus mesenteric vasculitis (LMV) is a rare but potentially life-threatening clinical entity in systemic lupus erythematosus (SLE) patients. Objective The present study was initiated to explore the clinical features and associated factors of LMV in SLE patients. Methods We conducted a retrospective study on 50 cases of SLE patients with lupus mesenteric vasculitis (LMV) from January 2010 to December 2019 and 89 cases of non-LMV-SLE patients with similar demographic and comorbidities were included as control. All the data regarding clinical features, laboratory findings, and treatment were reviewed independently by two experts in the field. Both univariate and multivariate logistic regression analyses were employed to identify the associated factors of LMV. Results The incidence of LMV was 2.9% among hospitalized SLE patients in the current study. The most frequent symptom and physical sign of LMV were respectively abdominal pain (48, 96%) and abdominal tenderness (45, 90%). Through univariate and subsequent multivariate analysis, oral ulcer (OR, 4.25; P = 0.024), urinary tract involvement (OR, 5.23; P = 0.021), and elevated D-dimer (OR, 1.121; P = 0.008) were demonstrated to be positively associated with LMV, while percentage of lymphocytes (OR, 0.928; P = 0.004) and complement 3 (OR, 0.048; P = 0.008) were negatively correlated with LMV. Conclusions Oral ulcer, urinary tract involvement, reduced percentage of lymphocytes and complement 3, elevated D-dimer could be associated factors for LMV in SLE patients.
Collapse
Affiliation(s)
- Hongxu Wang
- Department of Laboratory Medicine, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China
| | - Qing Gao
- Department of Gastroenterology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China
| | - Guanyi Liao
- Department of Gastroenterology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China
| | - Sirui Ren
- Department of Intensive Care Unit, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China
| | - Wenxian You
- Department of Gastroenterology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
| |
Collapse
|
|
37
|
Systemic manifestations - do not forget the small bowel. Curr Opin Gastroenterol 2021; 37:234-244. [PMID: 33606400 DOI: 10.1097/mog.0000000000000717] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
PURPOSE OF REVIEW Systemic diseases can afflict the small bowel (SB) but be challenging to diagnose. In this review, we aim to provide a broad overview of these conditions and to summarise their management. RECENT FINDINGS Small bowel capsule endoscopy (SBCE) is an important modality to investigate pathology in the SB. SB imaging can be complementary to SBCE for mural and extramural involvement and detection of multiorgan involvement or lymphadenopathy. Device assisted enteroscopy provides a therapeutic arm, to SBCE enabling histology and therapeutics to be carried out. SUMMARY SB endoscopy is essential in the diagnosis, management and monitoring of these multi-system conditions. Collaboration across SB centres to combine experience will help to improve the management of some of these rarer SB conditions.
Collapse
|
|
38
|
Ileocolic intussusception caused by mass-forming fibro-granulation from healed ulcer masquerading as small bowel lipoma. Clin J Gastroenterol 2021; 14:522-530. [PMID: 33405177 DOI: 10.1007/s12328-020-01329-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2020] [Accepted: 12/17/2020] [Indexed: 10/22/2022]
Abstract
Small bowel intussusception in adults is rare. Unlike children, adults with intussusception generally have a causative lead point, of which a majority is benign. We report the case of a 55-year-old woman with systemic lupus erythematosus on steroids and nonsteroidal anti-inflammatory drugs who presented with intermittent melena. Contrast computed tomography revealed intussusception of the terminal ileum, with a low density mass which had advanced into the cecum as the lead point. The patient was diagnosed with ileocolic intussusception. The mass was observed in the terminal ileum on colonoscopy, indicating spontaneous reduction. As endoscopic treatment did not appear feasible, laparoscopic small bowel resection was performed with no complications. The resected specimen revealed a pedunculated mass over a healed ulcer. Pathology showed a deep ulcer reaching the subserosa with fibro-granulation, with no evidence of mesenteric vasculitis, thrombus, bacteria, fungi, granulomas, lipoma, or other tumors. The patient was diagnosed with ileocolic intussusception due to a fibro-granulation mass formed on a healed ulcer. Based on the patient's systemic lupus erythematosus being well controlled, the absence of other causative factors, and the discovery of several small bowel erosions on subsequent capsule endoscopy, the ulcer was strongly suspected to be drug induced. Both steroids and nonsteroidal anti-inflammatory drugs were reduced and proton pump inhibitors were discontinued by her rheumatologist after surgery. No recurrence has been observed during 4 months of follow-up.
Collapse
|
|
39
|
Acharya N, Chattopadhyay A, Sinha A, Dhir V. Lupus with abdominal pain. Rheumatology (Oxford) 2021; 60:475. [PMID: 32734289 DOI: 10.1093/rheumatology/keaa384] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Affiliation(s)
- Nupoor Acharya
- Division of Rheumatology, Department of Internal Medicine
| | | | - Anindita Sinha
- Department of Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Varun Dhir
- Division of Rheumatology, Department of Internal Medicine
| |
Collapse
|
|
40
|
Management of Severe Refractory Systemic Lupus Erythematosus: Real-World Experience and Literature Review. Clin Rev Allergy Immunol 2020; 60:17-30. [PMID: 33159635 DOI: 10.1007/s12016-020-08817-2] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/27/2020] [Indexed: 12/11/2022]
Abstract
Systemic lupus erythematosus (SLE) is a highly heterogeneous disease affecting multiple organs and is characterized by an aberrant immune response. Although the mortality of SLE has decreased significantly since the application of glucocorticoids, severe or refractory SLE can potentially cause irreversible organ damage and contribute to the disease morbidity and mortality. Early recognition of severe SLE or life-threatening conditions is of great challenge to clinicians since the onset symptoms can be rapid and aggressive, involving the visceral organs of the neuropsychiatric, gastrointestinal, hematologic, renal, pulmonary, and cardiovascular systems, etc. Additionally, SLE patients with specific comorbidities and detrimental complications could lead to a clinical dilemma and contribute to poor prognosis. Prompt and adequate treatment for severe refractory SLE is crucial for a better prognosis. However, as evidence from well-designed randomized controlled trials is limited, this review aims to provide real-world evidence based on cohort studies from Peking Union Medical College Hospital, the national tertiary referral center in China, together with the literature, on clinical characteristics, risks and prognostic factors, and treatment strategies for severe and/or refractory SLE.
Collapse
|
|
41
|
Zhang X, Furth EE, Tondon R. Vasculitis Involving the Gastrointestinal System Is Often Incidental but Critically Important. Am J Clin Pathol 2020; 154:536-552. [PMID: 32789454 DOI: 10.1093/ajcp/aqaa083] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022] Open
Abstract
OBJECTIVES This study was aimed to investigate the significance of unexpected vasculitis identified in gastrointestinal (GI) specimens by determining its prevalence and correlation with clinical outcomes. METHODS GI specimens with histologic evidence of vasculitis were identified in our pathology database over a 10-year period (January 2008 to August 2018). Clinical history, treatment, and follow-up were reviewed. RESULTS Of the 131,367 GI pathology cases received over the 10-year study period, 29 (0.02%) cases showed histologic evidence of GI vasculitis. The majority (69%, 20/29) were not clinically suspected. Of these, 20% (4/20) of patients were subsequently diagnosed with systemic vasculitis. During the mean follow-up period of 34.0 months, 24% (4/17) of the patients with this unexpected diagnosis died as the result of direct complications of GI vasculitis. We also found that 95% of cases with unexpected vasculitis in their GI pathology specimens were communicated in a timely manner to the ordering physicians, which necessitated the immediate initiation of additional workups in 85% of these patients. CONCLUSIONS The GI involvement of vasculitis is rarely encountered by pathologists, but its diagnosis carries tremendous clinical significance with a high mortality rate. Therefore, timely communication is highly recommended for the early diagnosis and treatment of this disease.
Collapse
Affiliation(s)
- Xiaoming Zhang
- Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia
| | - Emma Elizabeth Furth
- Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia
| | - Rashmi Tondon
- Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia
| |
Collapse
|
|
42
|
Yoshida Y, Omoto T, Kohno H, Tokunaga T, Kuranobu T, Yukawa K, Watanabe H, Oi K, Sugimoto T, Mokuda S, Nojima T, Hirata S, Sugiyama E. Lower CH50 as a predictor for intractable or recurrent lupus enteritis: A retrospective observational study. Mod Rheumatol 2020; 31:643-648. [PMID: 32815450 DOI: 10.1080/14397595.2020.1812871] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
OBJECTIVES Lupus enteritis (LE) is a rare but well-known gastrointestinal manifestation of systemic lupus erythematosus (SLE). This study was conducted to identify prognostic factors associated with poor responses in patients with LE. METHODS We consecutively registered patients diagnosed with LE between January 2009 and October 2019, and retrospectively compared their clinical characteristics based on whether they had good or poor responses to treatment. RESULTS A total of 13 patients (17 episodes) were included. The median age was 41 years, and 12 patients were female. A comparison of clinical characteristics between groups revealed similar computed tomography (CT) findings. However, serum CH50 levels were significantly lower in the poor response group (median [interquartile ranges (IQR)]; 29.2 [25.3-46.9] U/mL vs 19.3 [7.8-24.0] U/mL, p = .0095). More patients in the poor response group had higher titers of anti-cardiolipin β2-glycoprotein I antibody (anti-CL β2GPI Ab) and were started on glucocorticoids (GCs) at moderate doses. In multivariable analysis, serum CH50 level was independently associated with poor response to induction therapy. CONCLUSION Lower levels of CH50 at the time of initial treatment predicted inadequate treatment response in patients with LE.
Collapse
Affiliation(s)
- Yusuke Yoshida
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Takuji Omoto
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan.,Department of Rheumatology, Hiroshima Prefectural Hospital, Hiroshima, Japan
| | - Hiroki Kohno
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Tadahiro Tokunaga
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Tatsuomi Kuranobu
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Kazutoshi Yukawa
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Hirofumi Watanabe
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Katsuhiro Oi
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Tomohiro Sugimoto
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Sho Mokuda
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Takaki Nojima
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Shintaro Hirata
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| | - Eiji Sugiyama
- Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan
| |
Collapse
|
|
43
|
Taibi A, Durand Fontanier S, Charissoux A, Mathonnet M. Man With Joint Pain and Abdominal Pain. Ann Emerg Med 2020; 76:153-190. [PMID: 32713480 DOI: 10.1016/j.annemergmed.2020.01.023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2019] [Indexed: 11/16/2022]
Affiliation(s)
- Abdelkader Taibi
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France; University Limoges, CNRS, XLIM, UMR 7252, Limoges, France
| | - Sylvaine Durand Fontanier
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France; University Limoges, CNRS, XLIM, UMR 7252, Limoges, France
| | | | - Muriel Mathonnet
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France
| |
Collapse
|
|
44
|
Chen Z, Zhou J, Li J, Zhou Y, Wang X, Li T, Gu L, Sun F, Wu W, Xu W, Sun S, Chen J, Li J, Lu L, Zhang W, Zhao Y, Ye S. Systemic lupus erythematosus gastrointestinal involvement: a computed tomography-based assessment. Sci Rep 2020; 10:6400. [PMID: 32286471 PMCID: PMC7156738 DOI: 10.1038/s41598-020-63476-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2019] [Accepted: 03/26/2020] [Indexed: 11/09/2022] Open
Abstract
Systemic lupus erythematosus (SLE) gastrointestinal (GI) complication is characterized by multi-segment and multi-compartment involvement. The aim of this study is to develop a computed tomography (CT) image-based system for disease evaluation. SLE patients with GI involvement from two independent cohorts were retrospectively included. Baseline abdominal CT scan with intravenous and oral contrast was obtained from each individual. A CT scoring system incorporating the extent of GI tract involvement and intestinal wall thickness, along with extra-GI compartment involvement, was developed and validated. The outcome measurement was the time to GI functional recovery, defined as the time to tolerable per os (PO) intake ≥50% of ideal calories (PO50). A total of 54 and 37 patients with SLE GI involvement were enrolled in the derivation and validation cohorts, respectively. The CT scores for SLE GI involvement were positively correlated with patients' time to PO50 (r = 0.57, p < 0.0001, derivation cohort; r = 0.42, p = 0.0093, validation cohort). Patients with a CT score ≤ 3 had a shorter time to PO50 (median time of 0 day) in pooled cohort, whereas those with a CT score > 3 incurred a significantly prolonged recovery with a median time to PO50 of 13 days (p < 0.0001). The CT-based scoring system may facilitate more accurate assessment and individualized management of SLE patients with GI involvement.
Collapse
Affiliation(s)
- Zhiwei Chen
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Jiaxin Zhou
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China
| | - Jiaoyu Li
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Yiquan Zhou
- 3Department of Clinical Nutrition, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200127, China
| | - Xiaodong Wang
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Ting Li
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Liyang Gu
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Fangfang Sun
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Wanlong Wu
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Wenwen Xu
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Shuhui Sun
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Jie Chen
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Jiajie Li
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Liangjing Lu
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China
| | - Wen Zhang
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China
| | - Yan Zhao
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.
| | - Shuang Ye
- Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiaotong University School of Medicine, Shanghai, 201112, China.
| |
Collapse
|
|
45
|
Kenar G, Atay K, Yüksek GE, Öz B, Koca SS. Gastrointestinal vasculitis due to systemic lupus erythematosus treated with rituximab: a case report. Lupus 2020; 29:640-643. [PMID: 32188302 DOI: 10.1177/0961203320910803] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Background Patients with systemic lupus erythematosus may present with variable gastrointestinal manifestations including peritonitis, pancreatitis, enteritis, and vasculitis. Gastrointestinal vasculitis is one of the most devastating complications of systemic lupus erythematosus, with a mortality rate of 50% when it progresses to bowel ischemia and is complicated by hemorrhage or perforation. Case report A young female patient known to have systemic lupus erythematosus, lupus nephritis, and antiphospholipid antibody syndrome, on immunosuppressive treatment presented to the emergency department with acute abdominal pain and nausea. The clinical findings were first associated with an acute flare of lupus nephritis according to the assessments with active laboratory parameters. However, over a short time the abdominal pain worsened and was accompanied by peritonitis and gastrointestinal hemorrhage. The computed tomography scans demonstrated a dilated bowel and thickening of the bowel compatible with gastrointestinal vasculitis. The upper and lower gastrointestinal endoscopy supported the diagnosis of vasculitis by showing multiple ulcerative lesions along the gastrointestinal tract. The patient was successfully treated with pulse corticosteroids urgently, with a fast response to subsequent rituximab therapy without any relapses. Treatment with cyclophosphamide was not preferred because the patient had a high cumulative dose. Conclusion Gastrointestinal vasculitis should be a primary differential diagnosis in patients with systemic lupus erythematosus presenting with abdominal pain because of its rapid progression and high mortality. The treatment choice has been suggested as cyclophosphamide for severe cases in the literature. In this case report, a patient successfully treated with rituximab without any relapses was documented.
Collapse
Affiliation(s)
- Gökçe Kenar
- Rheumatology department, Mardin State Hospital, Mardin, Turkey
| | - Kadri Atay
- Gastroenterology department, Mardin State Hospital, Mardin, Turkey
| | | | - Burak Öz
- Rheumatology department, Fırat University School of Medicine, Elazığ, Turkey
| | | |
Collapse
|
|
46
|
Wang Z, Wu C, Ruan F, Li Z, Peng X, Wang P. A case of systemic lupus erythematosus with rectal necrosis. Lupus 2020; 29:334-339. [PMID: 31924142 DOI: 10.1177/0961203319897130] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
Lupus mesenteric vasculitis is one of the most devastating complications of systemic lupus erythematosus (SLE) and may lead to a spectrum of complications, including ulceration, haemorrhage, bowel necrosis, perforation, serositis and ascites. Among such complications, intestinal necrosis and intestinal perforation are the most serious. Rectal necrosis is a rare manifestation of SLE, with only two case reports in the English literature. Here, we report the case of a 59-year-old male patient with SLE complicated by rectal necrosis that was initially misdiagnosed as acne and rectal tumours. After two surgeries and the addition of immunosuppressive therapy, the patient was eventually cured and discharged.
Collapse
Affiliation(s)
- Z Wang
- Emergency Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - C Wu
- Shangdong University, Jinan, China
| | - F Ruan
- Emergency Surgery, Linyi People's Hospital, Linyi, China
| | - Z Li
- Emergency Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - X Peng
- Emergency Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| | - P Wang
- Emergency Surgery, Affiliated Hospital of Qingdao University, Qingdao, China
| |
Collapse
|
|
47
|
Hadi YB, Lindsay J, Naqvi SFZ, Al-Jaroushi H. Systemic Lupus Erythematosus Presenting with Ischemic Proctitis and Abdominal Compartment Syndrome. Case Rep Gastrointest Med 2020; 2020:5723403. [PMID: 32099695 PMCID: PMC7040385 DOI: 10.1155/2020/5723403] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2019] [Accepted: 01/13/2020] [Indexed: 11/17/2022] Open
Abstract
Ischemic colitis and proctitis is a rare manifestation of systemic lupus erythematosus (SLE) and results from mesenteric vasculitis. Owing to diverse blood supply and presence of multiple collaterals, rectum is the least effected site in SLE enteritis. Ischemic proctocolitis as the presenting feature of SLE is exceedingly rare, with only three cases reported in the published scientific literature. We present the first case of SLE presenting as ischemic proctitis, leading to intraperitoneal hemorrhage and abdominal compartment syndrome. A young lady presented with ischemic proctitis and a hematoma masquerading as a pelvic mass, with subsequent development of massive intraperitoneal hemorrhage, shock, and rectal perforation. The patient required urgent surgery and was initiated on high-dose steroids.
Collapse
Affiliation(s)
| | - John Lindsay
- 2West Virginia University School of Medicine, Morgantown, WV, USA
| | | | - Hatim Al-Jaroushi
- 3Department of Medicine, Section of Pulmonary and Critical Care Medicine, West Virginia University, Morgantown, WV, USA
| |
Collapse
|
|
48
|
Smith EMD, Lythgoe H, Hedrich CM. Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus. Front Pediatr 2019; 7:149. [PMID: 31143758 PMCID: PMC6521594 DOI: 10.3389/fped.2019.00149] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2018] [Accepted: 04/01/2019] [Indexed: 01/08/2023] Open
Abstract
Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.
Collapse
Affiliation(s)
- Eve M D Smith
- Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.,Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom
| | - Hanna Lythgoe
- St Helen's and Knowsley Teaching Hospital NHS Trust, St Helens, United Kingdom
| | - Christian M Hedrich
- Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.,Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom
| |
Collapse
|
|
49
|
Maruyama A, Nagashima T, Iwamoto M, Minota S. Clinical characteristics of lupus enteritis in Japanese patients: the large intestine-dominant type has features of intestinal pseudo-obstruction. Lupus 2018; 27:1661-1669. [PMID: 30028259 DOI: 10.1177/0961203318785770] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
This study was performed to investigate the clinical characteristics of lupus enteritis in Japanese patients with systemic lupus erythematosus (SLE). A total of 481 patients with SLE admitted to our hospital between 2001 and 2015 were retrospectively reviewed. Diagnosis of lupus enteritis was based on the following three criteria: (1) abdominal symptoms, (2) diffuse long-segment bowel thickening and (3) a requirement for glucocorticoid therapy. Lupus enteritis was identified in 17 patients (3.5%) and there were two distinct types: small intestine-dominant and large intestine-dominant. Significant differences between the two types were noted with respect to the age, frequency of biopsy-proven lupus nephritis, frequency of rectal involvement, maximum bowel wall thickness, and requirement for steroid pulse therapy. Among patients with large intestine-dominant lupus enteritis, 60% had extra-intestinal symptoms (hydroureter, bladder wall thickening, and bile duct dilatation) that are known complications of intestinal pseudo-obstruction. Two patients with large intestine-dominant lupus enteritis developed intestinal pseudo-obstruction either before or after diagnosis of lupus enteritis. Five patients (29%) developed recurrence during a median observation period of 7.2 years (1.4-14.4 years). In conclusion, large intestine-dominant lupus enteritis resembles intestinal pseudo-obstruction and these two diseases may have a common pathogenesis.
Collapse
Affiliation(s)
- A Maruyama
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - T Nagashima
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - M Iwamoto
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - S Minota
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| |
Collapse
|
|
50
|
Iwasaki K, Morimoto M, Ota G, Koinuma K, Horie H, Sata N, Nakaya T. Partial small intestinal resection for successful surgical management of refractory protein-losing gastroenteropathy in systemic lupus erythematosus: A case report and literature review. Medicine (Baltimore) 2018; 97:e11357. [PMID: 30045258 PMCID: PMC6078739 DOI: 10.1097/md.0000000000011357] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Although systemic lupus erythematosus (SLE) can be complicated by various gastrointestinal tract diseases, it is rarely associated with lupus enteritis and protein-losing enteropathy (PLE). We report here the successful surgical treatment of lupus enteritis and therapy-resistant and refractory PLE in a patient with SLE. We also provide a review of relevant literature. PATIENT CONCERNS A 16-year-old girl presenting with polyarthritis, malar rash, and palmar erythema was indicated for steroid therapy on the basis of positive results for antinuclear, anti-Smith, and antiphospholipid antibodies, which confirmed the diagnosis of SLE. During the course of steroid therapy, the patient developed acute abdomen and hypoalbuminemia. DIAGNOSES Computed tomography and Tc-labeled human serum albumin scintigraphy revealed abnormal findings, and a diagnosis of lupus enteritis and PLE was made. Steroid treatment was continued but no significant improvement was observed, and the patient was referred and admitted to our hospital. Double-balloon enteroscopy revealed multiple ischemic stenoses and mucosal necroses in the small intestine, suggesting that PLE was associated with ischemic enteritis due to antiphospholipid syndrome. The patient received steroids, immunosuppressive drugs, and antithrombotic therapy, with no improvement in symptoms. Thus, the disease was judged to be refractory and resistant to medical therapy, and the patient was indicated for surgical treatment. INTERVENTIONS Partial small intestinal resection was performed by removing the segment of the small intestine presenting PLE lesions, and a double-end ileostomy was created. OUTCOMES Multiple stenotic lesions were confirmed in the resected segment. Histopathology evaluation revealed marked inflammatory cell infiltration in the intestinal tract wall and recanalization of the vessels, suggesting a circulatory disorder caused by vasculitis and antiphospholipid syndrome. Postoperatively, the clinical course was good. Serum albumin levels and body weight increased as nutritional status improved significantly. Secondary enteroenterostomy with ileostomy closure could be performed at 2 months after the initial surgery. LESSONS Timely surgical treatment can be successful in managing therapy-resistant and refractory PLE in patients with SLE.
Collapse
Affiliation(s)
| | | | | | | | | | | | - Takeo Nakaya
- Department of Pathology, Jichi Medical University, Tochigi, Japan
| |
Collapse
|