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Inomata K, Takasawa E, Mieda T, Iizuka Y, Chikuda H. Late-Onset Esophageal Perforation After Salvage Anterior Cervical Spine Surgery in a Patient With Systemic Sclerosis. Cureus 2023; 15:e37831. [PMID: 37214044 PMCID: PMC10198659 DOI: 10.7759/cureus.37831] [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: 04/19/2023] [Indexed: 05/23/2023] Open
Abstract
Systemic sclerosis (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis that results in progressive organ dysfunction including the esophagus. We herein report our experience of a patient with SSc who underwent salvage anterior cervical spine surgery that led to late-onset esophageal perforation. A 57-year-old female had progressive cervical kyphosis after laminoplasty for cervical spondylotic myelopathy. We performed anterior cervical discectomy and fusion using a stand-alone cage. Despite prolonged use of a neck collar, migration of the anterior cage was noted three months after surgery. Rapid progression of kyphotic deformity prompted us to perform revision surgery of circumferential cervical correction. However, conventional posterior surgery was precluded due to the extremely poor condition of her neck, including severely sclerotic skin and atrophic muscles. To address this, she underwent a posterior fusion with the closed technique and C4-C5 corpectomy and bone grafting using a low-profile anterior plate. One year after the surgery, CT and routine upper gastrointestinal endoscopy (UGE) showed no evidence of esophagus injury. She remained asymptomatic thereafter. Over three years after her last surgery, however, follow-up CT incidentally revealed an abnormal air leak around the anterior plate. UGE demonstrated large esophagus perforation with the exposed metal plate. As she had already been on parenteral nutrition due to the disease progression of SSc, we decided not to remove the implant. Potential esophagus perforation after anterior cervical spine surgery, even years later, should be considered regardless of the patient's symptoms, such as chest pain and dysphagia. Spine surgeons need to be cognizant of the fragility of the esophagus, especially in patients with SSc. For patients with SSc, posterior reconstruction alone is recommended as a relatively safe option, even with a suboptimal skin condition.
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Affiliation(s)
- Kazuhiro Inomata
- Department of Orthopedic Surgery, Gunma University, Maebashi, JPN
| | - Eiji Takasawa
- Department of Orthopedic Surgery, Gunma University, Maebashi, JPN
| | - Tokue Mieda
- Department of Orthopedic Surgery, Gunma University, Maebashi, JPN
| | - Yoichi Iizuka
- Department of Orthopedic Surgery, Gunma University, Maebashi, JPN
| | - Hirotaka Chikuda
- Department of Orthopedic Surgery, Gunma University, Maebashi, JPN
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Arar AM, DeLay K, Leiman DA, Menard-Katcher P. Esophageal Manifestations of Dermatological Diseases, Diagnosis and Management. CURRENT TREATMENT OPTIONS IN GASTROENTEROLOGY 2022; 20:513-528. [PMID: 37287750 PMCID: PMC10243751 DOI: 10.1007/s11938-022-00399-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 08/29/2022] [Indexed: 06/09/2023]
Abstract
Purpose of Review The purpose of this article is to discuss the diagnosis and treatment of diseases that affect both the skin and the esophagus. Recent Findings The diagnosis of dermatological conditions that affect the esophagus often requires endoscopy and biopsy with some conditions requiring further investigation with serology, immunofluorescence, manometry, or genetic testing. Many conditions that affect the skin and esophagus can be treated successfully with systemic steroids and immunosuppressants including pemphigus, pemphigoid, HIV, esophageal lichen planus, and Crohn's disease. Many conditions are associated with esophageal strictures which are treated with endoscopic dilation. Furthermore, many of the diseases are pre-malignant and require vigilance and surveillance endoscopy. Summary Diseases that affect the skin and esophagus can be grouped by their underlying etiology: autoimmune (scleroderma, dermatomyositis, pemphigus, pemphigoid), infectious (herpes simplex virus, cytomegalovirus, human immunodeficiency virus), inflammatory (lichen planus and Crohn's disease), and genetic (epidermolysis bullosa, Cowden syndrome, focal dermal hypoplasia, and tylosis). It is important to consider primary skin conditions that affect the esophagus when patients present with dysphagia of unknown etiology and characteristic skin findings.
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Affiliation(s)
- Amr M. Arar
- Department of Internal Medicine, University of Colorado School of Medicine - Anschutz Medical Campus, Denver, CO, USA
| | - Kelli DeLay
- Division of Gastroenterology and Hepatology, University of Colorado School of Medicine - Anschutz Medical Campus, Denver, CO, USA
| | - David A. Leiman
- Division of Gastroenterology, Duke University Medical Center, Durham NC, USA
- Duke Clinical Research Institute, Durham NC, USA
| | - Paul Menard-Katcher
- Division of Gastroenterology and Hepatology, University of Colorado School of Medicine - Anschutz Medical Campus, Denver, CO, USA
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Kadakuntla A, Juneja A, Sattler S, Agarwal A, Panse D, Zakhary N, Pasumarthi A, Shapiro L, Tadros M. Dysphagia, reflux and related sequelae due to altered physiology in scleroderma. World J Gastroenterol 2021; 27:5201-5218. [PMID: 34497445 PMCID: PMC8384755 DOI: 10.3748/wjg.v27.i31.5201] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 05/13/2021] [Accepted: 07/30/2021] [Indexed: 02/06/2023] Open
Abstract
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
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Affiliation(s)
| | - Ankit Juneja
- Albany Medical College, Albany, NY 12208, United States
| | | | | | - Drishti Panse
- Albany Medical College, Albany, NY 12208, United States
| | - Nardin Zakhary
- Department of Dentistry, Ministry of Health, Alexandria 21500, Egypt
| | | | - Lee Shapiro
- Division of Rheumatology, Albany Medical Center, Albany, NY 12208, United States
| | - Micheal Tadros
- Division of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States
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4
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Kadakuntla A, Juneja A, Sattler S, Agarwal A, Panse D, Zakhary N, Pasumarthi A, Shapiro L, Tadros M. Dysphagia, reflux and related sequelae due to altered physiology in scleroderma. World J Gastroenterol 2021; 27:5201-5218. [PMID: 34497445 DOI: 10.3748/wjg.v27.i31.5201.pmid:] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 05/13/2021] [Accepted: 07/30/2021] [Indexed: 01/30/2025] Open
Abstract
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
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Affiliation(s)
| | - Ankit Juneja
- Albany Medical College, Albany, NY 12208, United States
| | | | | | - Drishti Panse
- Albany Medical College, Albany, NY 12208, United States
| | - Nardin Zakhary
- Department of Dentistry, Ministry of Health, Alexandria 21500, Egypt
| | | | - Lee Shapiro
- Division of Rheumatology, Albany Medical Center, Albany, NY 12208, United States
| | - Micheal Tadros
- Division of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States.
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Voulgaris TA, Karamanolis GP. Esophageal manifestation in patients with scleroderma. World J Clin Cases 2021; 9:5408-5419. [PMID: 34307594 PMCID: PMC8281422 DOI: 10.12998/wjcc.v9.i20.5408] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2021] [Revised: 04/22/2021] [Accepted: 05/10/2021] [Indexed: 02/06/2023] Open
Abstract
The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis (SSc). Esophageal involvement may lead to a significant reduction in patient quality of life. The exact pathophysiology is complex and not yet fully elucidated. Ultimately, esophageal smooth muscle becomes atrophied and replaced by fibrous tissue leading to severe motility disturbance of the distal esophagus. Symptoms are mainly attributed to gastroesophageal reflux disease and to esophageal dysmotility. Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease. No formed guidelines exist about the diagnostic modalities used to assess esophageal disease in patients with SSc, though upper gastrointestinal endoscopy is the first and most important modality used as it can reveal alterations commonly observed in patients with SSc. Further exploration can be made by high resolution manometry and pH-impedance study. Proton pump inhibitors remain the mainstay of treatment, while prokinetic agents are commonly used as add-on therapy in patients with symptoms attributed to gastroesophageal reflux disease not responding to standard therapy as well as to motility disturbances. Gastroesophageal reflux disease symptoms in patients with SSc are frequently difficult to manage, and new therapeutic modalities are emerging. The role of surgical treatment is restricted and should only be preserved for resistant cases.
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Affiliation(s)
- Theodoros A Voulgaris
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
| | - Georgios P Karamanolis
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
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Nakane S, Umeda M, Kawashiri SY, Mukaino A, Ichinose K, Higuchi O, Maeda Y, Nakamura H, Matsuo H, Kawakami A. Detecting gastrointestinal manifestations in patients with systemic sclerosis using anti-gAChR antibodies. Arthritis Res Ther 2020; 22:32. [PMID: 32085768 PMCID: PMC7035754 DOI: 10.1186/s13075-020-2128-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2019] [Accepted: 02/12/2020] [Indexed: 12/18/2022] Open
Abstract
BACKGROUND Patients with systemic sclerosis (SSc) complicated by gastrointestinal dysmotility are difficult to treat and have high mortality. To clarify the pathogenesis of gastrointestinal manifestations, we aimed to demonstrate the association among the clinical features of SSc, the serological markers, the autoantibodies against nicotinic acetylcholine receptor at autonomic ganglia (gAChR). METHODS Fifty patients were enrolled and divided into two groups according to the presence or absence of gastrointestinal manifestations, and the characteristics were analyzed between these two groups. We measured biomarkers and the autoantibodies against two gAChRα3 and β4 subunits to test sera samples. Furthermore, patients were classified based on the presence or absence of anti-gAChR autoantibodies, and their clinical features were compared. RESULTS In patients with SSc and gastrointestinal manifestations, digital ulcers were more frequent (p = 0.050) and VEGF expression was significantly higher (p = 0.038). Seven subjects with SSc were seropositive for α3 subunit, whereas one patient was seropositive for β4 subunit. The mean level of anti-gAChRα3 autoantibodies in SSc patients with gastrointestinal manifestations was significantly higher than that in SSc patients without gastrointestinal manifestations (p = 0.001). The group of patients with SSc and gAChR autoantibodies had significantly higher endostatin levels (p = 0.046). CONCLUSIONS This study is the first to demonstrate that clinical characteristics of SSc patients with seropositivity for gAChR autoantibodies. Patients with SSc have circulating autoantibodies against gAChR, which may contribute to gastrointestinal manifestations associated with this disease, suggesting that gAChR-mediated autonomic neurotransmission may provide a pathomechanism for gastrointestinal dysmotility in SSc.
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Affiliation(s)
- Shunya Nakane
- Department of Neuroimmunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
- Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan
- Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan
- Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, 1-1-1, Honjo, Chuouku, Kumamoto-shi, Kumamoto, 860-8556 Japan
| | - Masataka Umeda
- Department of Immunology and Rheumatology, Unit of Translational Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
| | - Shin-ya Kawashiri
- Department of Immunology and Rheumatology, Unit of Translational Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
| | - Akihiro Mukaino
- Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, 1-1-1, Honjo, Chuouku, Kumamoto-shi, Kumamoto, 860-8556 Japan
- Department of Neurology and Strokology, Nagasaki University Hospital, Nagasaki, Japan
| | - Kunihiro Ichinose
- Department of Immunology and Rheumatology, Unit of Translational Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
| | - Osamu Higuchi
- Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan
| | - Yasuhiro Maeda
- Department of Neuroimmunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
- Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan
- Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan
| | - Hideki Nakamura
- Department of Immunology and Rheumatology, Unit of Translational Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
| | - Hidenori Matsuo
- Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan
| | - Atsushi Kawakami
- Department of Immunology and Rheumatology, Unit of Translational Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan
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Yang H, Xu D, Li MT, Yao Y, Jin M, Zeng XF, Qian JM. Gastrointestinal manifestations on impaired quality of life in systemic sclerosis. J Dig Dis 2019; 20:256-261. [PMID: 30838807 DOI: 10.1111/1751-2980.12720] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2018] [Revised: 09/20/2018] [Accepted: 02/25/2019] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To analyze the impact of gastrointestinal manifestations on quality of life in patients with systemic sclerosis (SSc) and to provide clinical evidence for their early treatment and health-related management. METHODS Altogether 65 patients admitted to the Peking Union Medical College Hospital selected from a disease cohort and 127 matched controls were enrolled. A self-assessment questionnaire was completed by all participants. Each participant completed scleroderma gastrointestinal tract 1.0 (SSC-GIT 1.0) questionnaire (including reflux or indigestion, diarrhea, constipation, distention, emotional well-being, and social functioning). Autoimmune antibodies were tested in SSc patients. RESULTS Among these SSc cases, gastrointestinal manifestations were seen in 84.6%. Reflux/indigestion and diarrhea were more common in SSc patients than in the control group (67.7% vs 27.8%; 27.7% vs 10.2%, P < 0.05). SSc patients had a significantly higher percentage of abnormal social functioning than the control group (33.8% vs 3.9%, P < 0.05). SSc patients with abnormal social functioning and abnormal emotional well-being had worse distention, diarrhea, and constipation statuses. Patients with reflux or indigestion and diarrhea had lower anti-Scl-70 level than those without (both P < 0.05). Patients with distention had higher levels of anti-RNP and anti-SSA than those without distention (both P < 0.05). Patients with diarrhea had higher levels of anti-RNP than those without diarrhea (P = 0.014). CONCLUSIONS Gastrointestinal involvement is frequent in SSc, with reflux or indigestion as the most common symptom. The impaired quality of life in patients with SSc indicates that early and active management should be considered.
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Affiliation(s)
- Hong Yang
- Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Dong Xu
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Meng Tao Li
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Yao Yao
- Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Meng Jin
- Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Xiao Feng Zeng
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Jia Ming Qian
- Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
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Carvajal Alegria G, Gazeau P, Hillion S, Daïen CI, Cornec DYK. Could Lymphocyte Profiling be Useful to Diagnose Systemic Autoimmune Diseases? Clin Rev Allergy Immunol 2018; 53:219-236. [PMID: 28474288 DOI: 10.1007/s12016-017-8608-5] [Citation(s) in RCA: 33] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Considering the implications of B, T, and natural killer (NK) cells in the pathophysiology of systemic autoimmune diseases, the assessment of their distribution in the blood could be helpful for physicians in the complex process of determining a precise diagnosis. In primary Sjögren's syndrome, transitional and active naive B cells are increased and memory B cells are decreased compared to healthy controls and other systemic diseases. However, their utility to improve the accuracy of classification criteria has not been proven. In early untreated rheumatoid arthritis, proportions of regulatory T cells are constantly reduced, but other patterns are difficult to determine given the heterogeneity of published studies. In systemic lupus erythematosus, the lack of studies using large cohorts of patients and the diversity of the possible pathological mechanisms involved are also important impediments. Nevertheless, transitional B cell and plasma cell proportions are increased in most of the studies, the CD4/CD8 ratio is decreased, and the number of NK cells is reduced. Despite the low number of studies, anomalies of lymphocyte subset distribution was also described in ANCA-associated vasculitis, systemic scleroderma, and myositis. For now, flow cytometric analysis of lymphocyte subsets has focused mainly on specific subpopulations and is more useful for basic and translational research than for diagnostics in clinical practice. However, new modern methods such as mass cytometry and bioinformatics analyses may offer the possibility to simultaneously account for the relative proportions of multiple lymphocyte subsets and define a global profile in homogeneous groups of patients. The years to come will certainly incorporate such global lymphocyte profiling in reclassification of systemic autoimmune diseases.
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Affiliation(s)
- Guillermo Carvajal Alegria
- Service de Rhumatologie, Hôpital de la Cavale Blanche, CHRU Brest, BP 824, 29609, Brest cedex, France.,INSERM U1227, European University of Brest, Brest, France
| | - Pierre Gazeau
- Service de Rhumatologie, Hôpital de la Cavale Blanche, CHRU Brest, BP 824, 29609, Brest cedex, France
| | - Sophie Hillion
- INSERM U1227, European University of Brest, Brest, France.,Laboratoire d'Immunologie et Immunothérapie, CHRU Morvan, Brest, France
| | - Claire I Daïen
- Rheumatology Department, Lapeyronie Hospital and Montpellier I University, Montpellier, France.,UMR5535, CNRS, Institute of molecular genetic, Montpellier, France
| | - Divi Y K Cornec
- Service de Rhumatologie, Hôpital de la Cavale Blanche, CHRU Brest, BP 824, 29609, Brest cedex, France. .,INSERM U1227, European University of Brest, Brest, France.
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McFarlane IM, Bhamra MS, Kreps A, Iqbal S, Al-Ani F, Saladini-Aponte C, Grant C, Singh S, Awwal K, Koci K, Saperstein Y, Arroyo-Mercado FM, Laskar DB, Atluri P. Gastrointestinal Manifestations of Systemic Sclerosis. ACTA ACUST UNITED AC 2018; 8. [PMID: 30057856 PMCID: PMC6059963 DOI: 10.4172/2161-1149.1000235] [Citation(s) in RCA: 68] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative alterations of the microvasculature leading to fibrosis and loss of function of the skin and internal organs. Gastrointestinal manifestations of SSc are the most commonly encountered complications of the disease affecting nearly 90% of the SSc population. Among these complications, the esophagus and the anorectum are the most commonly affected. However, this devastating disorder does not spare any part of the gastrointestinal tract (GIT), and includes the oral cavity, esophagus, stomach, small and large bowels as well as the liver and pancreas. In this review, we present the current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways. We also discuss the clinical presentation and diagnosis of each part of the GIT affected by SSc. Finally, we highlight the latest developments in the management of this disease, addressing the severe malnutrition that affects this vulnerable patient population and ways to assess and improve the nutritional status of the patients.
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Affiliation(s)
- Isabel M McFarlane
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Manjeet S Bhamra
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Alexandra Kreps
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Sadat Iqbal
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Firas Al-Ani
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Carla Saladini-Aponte
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Christon Grant
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Soberjot Singh
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Khalid Awwal
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Kristaq Koci
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Yair Saperstein
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Fray M Arroyo-Mercado
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Derek B Laskar
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Purna Atluri
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
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10
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Systemic sclerosis and the gastrointestinal tract. GASTROENTEROLOGY REVIEW 2017; 12:163-168. [PMID: 29123575 PMCID: PMC5672703 DOI: 10.5114/pg.2017.70467] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/25/2017] [Accepted: 06/01/2017] [Indexed: 02/07/2023]
Abstract
Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. The disease results in heterogeneous clinical findings and different courses. Systemic sclerosis usually begins with the onset of Raynaud’s phenomenon (RP), followed by skin sclerosis and internal organ involvement, although it may appear synchronously with RP. Gastrointestinal involvement is a serious and prevalent complication of SSc, and the oesophagus is the most frequently affected organ. Both limited and diffuse cutaneous SSc involve internal organs, with the involvement of the gastrointestinal tract as a leading cause of morbidity. At present, treatment is mainly symptomatic with no disease-modifying drugs.
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