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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.
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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
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Chaparro CA, Bernal-Macías S, Muñoz OM. Lupus enteritis as systemic lupus erythematosus main manifestation: Two case reports. SAGE Open Med Case Rep 2024; 12:2050313X241247433. [PMID: 38628859 PMCID: PMC11020699 DOI: 10.1177/2050313x241247433] [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: 10/08/2023] [Accepted: 03/28/2024] [Indexed: 04/19/2024] Open
Abstract
Lupus enteritis denotes inflammation of the intestinal walls resulting from the influence of systemic lupus erythematosus. It represents a rare manifestation associated with notable morbidity and mortality, marked by nonspecific gastrointestinal symptoms. In this article, we present two cases of individuals experiencing severe gastrointestinal symptoms. They had a personal or familial history of autoimmunity with intestinal involvement consistent with the presentation of lupus enteritis. Following treatment with glucocorticoids and immunomodulators, both patients exhibited a satisfactory clinical evolution. While lupus enteritis remains an uncommon occurrence, its clinical significance is undeniable. Hence, it is imperative to maintain a high level of clinical suspicion to facilitate prompt diagnosis and treatment.
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Affiliation(s)
- Camilo Andrés Chaparro
- Internal Medicine Department, Hospital Universitario San Ignacio, Bogotá, Colombia
- Faculty of Medicine, Pontificia Universidad Javeriana, Bogotá, Colombia
| | - Santiago Bernal-Macías
- Faculty of Medicine, Pontificia Universidad Javeriana, Bogotá, Colombia
- Rheumatology Department, San Ignacio University Hospital, Bogotá, Colombia
| | - Oscar Mauricio Muñoz
- Internal Medicine Department, Hospital Universitario San Ignacio, Bogotá, Colombia
- Faculty of Medicine, Pontificia Universidad Javeriana, Bogotá, Colombia
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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.
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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
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Bindukumari Sureshkumar N, Gopinath P, Joshi A, Alumparambil Surendran S. Recurrent Lupus Enteritis While on Chronic Immunosuppressant Therapy. Cureus 2022; 14:e30149. [PMID: 36397920 PMCID: PMC9646243 DOI: 10.7759/cureus.30149] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/10/2022] [Indexed: 11/07/2022] Open
Abstract
Lupus enteritis is a poorly studied cause of abdominal pain in patients with systemic lupus erythematosus (SLE). We present the case of a 28-year-old female with a history of SLE for nine years. She has been on chronic immunosuppressant therapy for the last nine years due to an episode of lupus enteritis in the past. Currently, the patient presented to urgent care with a three-day history of waxing and waning symptoms of abdominal pain, vomiting, and diarrhea. In addition, the patient had skin rashes. Laboratory work was significant for leukopenia, hypocomplementemia, hematuria, and proteinuria. CT of the abdomen showed bowel thickening involving the entire ileum, distal jejunum, and first portion of the duodenum. It was accompanied by moderate mesenteric edema and a small amount of ascites. Since the patient was on long-term immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil, infectious etiology was of high consideration; however, it was ruled out after further testing. Along with continuing her home dose of mycophenolate mofetil and hydroxychloroquine, the patient was started on IV methylprednisolone 1 mg/kg for three days. The patient dramatically responded to IV steroids. The patient was transitioned to oral prednisone 60 mg daily, and steroids were tapered off by 10 mg each week. A repeat CT scan in two months showed the resolution of the previously visualized small bowel wall thickening. This case highlights that chronic immunosuppression should not preclude differential or diagnosis of lupus enteritis in a patient with a history of SLE.
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Radiographic Findings of Lupus Related Entero-colitis. Clin Rheumatol 2022; 41:1617-1618. [DOI: 10.1007/s10067-022-06112-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2021] [Revised: 02/16/2022] [Accepted: 02/17/2022] [Indexed: 11/03/2022]
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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: 1] [Impact Index Per Article: 0.3] [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.
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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.
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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: 24] [Impact Index Per Article: 6.0] [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.
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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
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Lupus Enteritis—an Unusual Cause for Acute Abdominal Pain. Indian J Surg 2021. [DOI: 10.1007/s12262-020-02481-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022] Open
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Garcelon N, Neuraz A, Benoit V, Salomon R, Burgun A. Improving a full-text search engine: the importance of negation detection and family history context to identify cases in a biomedical data warehouse. J Am Med Inform Assoc 2017; 24:607-613. [PMID: 28339516 DOI: 10.1093/jamia/ocw144] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2016] [Accepted: 08/31/2016] [Indexed: 12/19/2022] Open
Abstract
Objective The repurposing of electronic health records (EHRs) can improve clinical and genetic research for rare diseases. However, significant information in rare disease EHRs is embedded in the narrative reports, which contain many negated clinical signs and family medical history. This paper presents a method to detect family history and negation in narrative reports and evaluates its impact on selecting populations from a clinical data warehouse (CDW). Materials and Methods We developed a pipeline to process 1.6 million reports from multiple sources. This pipeline is part of the load process of the Necker Hospital CDW. Results We identified patients with "Lupus and diarrhea," "Crohn's and diabetes," and "NPHP1" from the CDW. The overall precision, recall, specificity, and F-measure were 0.85, 0.98, 0.93, and 0.91, respectively. Conclusion The proposed method generates a highly accurate identification of cases from a CDW of rare disease EHRs.
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Affiliation(s)
- Nicolas Garcelon
- Institut Imagine, Paris Descartes Université Paris Descartes-Sorbonne Paris Cité, Paris, France
- INSERM, Centre de Recherche des Cordeliers, UMR 1138 Equipe 22, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
| | - Antoine Neuraz
- Institut Imagine, Paris Descartes Université Paris Descartes-Sorbonne Paris Cité, Paris, France
- INSERM, Centre de Recherche des Cordeliers, UMR 1138 Equipe 22, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
| | - Vincent Benoit
- Institut Imagine, Paris Descartes Université Paris Descartes-Sorbonne Paris Cité, Paris, France
| | - Rémi Salomon
- Institut Imagine, Paris Descartes Université Paris Descartes-Sorbonne Paris Cité, Paris, France
- Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Assistance Publique -Hôpitaux de Paris (AP-HP), Université Paris Descartes, Sorbonne Paris Cité, France
| | - Anita Burgun
- INSERM, Centre de Recherche des Cordeliers, UMR 1138 Equipe 22, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
- Hôpital Européen Georges Pompidou, Assistance Publique -Hôpitaux de Paris (AP-HP), Université Paris Descartes, Sorbonne Paris Cité, France
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Müller-Ladner U. Gut and Liver in Vasculitic Disorders. Dig Dis 2016; 34:546-51. [PMID: 27333193 DOI: 10.1159/000445260] [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: 02/02/2023]
Abstract
BACKGROUND Although the gastrointestinal (GI) tract including its related organs is not generally regarded as one of the primary organ systems of primary and secondary vasculitic disorders, there are numerous mechanisms of these diseases operative in or around the different structures and compartments of the GI tract. KEY MESSAGES A majority of the respective clinical symptoms and problems are linked to an alteration of (peri)vascular homeostasis. Alteration of perivascular matrix metabolism can also affect the functional integrity and motility of the GI tract. Apart from the specific GI phenomena of the individual diseases as outlined in detail in this review, the epidemiology of GI involvement follows in general the characteristics of the respective underlying systemic disease. In addition, gender and age do neither influence the occurrence nor the severity of the GI manifestations significantly. With respect to clinical symptoms, vasculitides may result in abdominal pain, bleeding, ileus, intestinal necrosis and hematochezia because of reduced blood flow and hyper-acute occlusion in the antiphospholipid syndrome. Small-bowel involvement in vasculitic entities can cause pseudoobstruction, obstruction, malabsorption and bacterial overgrowth. Laboratory parameters can point to specific diseases but are frequently nonspecific. Thus, if biopsy fails or in unclear endoscopic situations, a variety of imaging techniques including Doppler ultrasound, abdominal CT, MRI and angiography are used and required for identification and localization of the underlying disease. Therapeutic strategies in vasculitides usually include corticosteroids and immunosuppressants, for example, cyclophosphamide in granulomatosis with polyangiitis and in panarteriitis nodosa but also biologics such as rituximab in ANCA-associated vasculitides. Virostatic drugs including interferon-α and ribavirin can be used in hepatitis B- and C-triggered vasculitides such as panarteriitis nodosa and hepatitis C-associated cryoglobulinemia. CONCLUSIONS Immediate diagnostic and therapeutic steps of action need to be performed if vasculitis of the GI tract is suspected in order to avoid irreversible damage to organs and to improve the well-being and life of the affected patient.
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Affiliation(s)
- Ulf Müller-Ladner
- Department of Rheumatology and Clinical Immunology, Kerckhoff-Klinik, Justus-Liebig University Giessen, Bad Nauheim, Germany
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Miranda-Bautista J, Fernández-Simón A, Pérez-Sánchez I, Menchén L. Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. World J Gastroenterol 2015; 21:5417-5420. [PMID: 25954116 PMCID: PMC4419083 DOI: 10.3748/wjg.v21.i17.5417] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/12/2014] [Revised: 11/12/2014] [Accepted: 12/22/2014] [Indexed: 02/07/2023] Open
Abstract
Weber-Christian disease (WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation, being the subcutaneous adipose tissue the most frequent one, followed by liver, spleen, bone marrow and mesenteric adipose tissue. Systemic corticosteroids are first line treatment, but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation. We report herein a case with WCD showing orbital, mesenteric and ileocolonic involvement, which required surgical treatment and also developed postoperative recurrence. Symptoms were resolved by administration of thalidomide and, afterwards, infliximab. To our knowledge, this is the first report of Weber-Christian disease with luminal ileocolonic involvement, treated with infliximab.
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