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Hongkanjanapong S, Pongkulkiat P, Mahakkanukrauh A, Suwannaroj S, Foocharoen C. Clinical outcomes and associated factors with mortality in systemic sclerosis patients with sarcopenia. Am J Med Sci 2025; 369:35-43. [PMID: 39033816 DOI: 10.1016/j.amjms.2024.07.025] [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: 04/30/2024] [Revised: 07/09/2024] [Accepted: 07/16/2024] [Indexed: 07/23/2024]
Abstract
BACKGROUND Despite the high incidence of sarcopenia in systemic sclerosis (SSc) patients, there is currently limited evidence on their outcomes. OBJECTIVES Our study aimed to determine clinical courses, outcomes, and identify factors associated with mortality in the SSc patients with sarcopenia. METHODS A historical cohort study was conducted in 180 adult SSc patients diagnosed with sarcopenia according to the criteria of Asian Working Group for Sarcopenia 2019, who were attending the Scleroderma Clinic at Khon Kaen University between July 2019 and November 2021. RESULTS Forty-one were diagnosed with sarcopenia. A total of 443.8 persons-year, the respective mortality rate for SSc patients with and without sarcopenia was 5.05 and 5.22 per 100-person-years, showing no statistical difference (p = 0.58). Sarcopenia was not a significant mortality risk in SSc patients with a hazard ratio (HR) of 1.34, 95% CI 0.48-3.75. The survival rate from the baseline evaluation of sarcopenia to the last follow-up of the patients with sarcopenia at 6-, 12-, 18-, and 24-months were 97.6%, 95.1%, 92.7%, and 87.8%. Hospitalization was the sole factor significantly associated with the mortality risk, with a HR of 14.21 (95% CI 2.36-85.60). Sarcopenia itself did not appear to be a significant predictor of disease progression, it did contribute significantly to the progression of salt and pepper skin (p=0.01). CONCLUSIONS The mortality rate of SSc patients with sarcopenia increased after a 2-year follow-up but no difference from non-sarcopenic patients. Once these patients required hospitalization, the mortality risk increased by over 10 times. Further long-term follow-up in a large cohort is suggested.
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Affiliation(s)
- Sirada Hongkanjanapong
- Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand
| | - Patnarin Pongkulkiat
- Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand
| | - Ajanee Mahakkanukrauh
- Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand
| | - Siraphop Suwannaroj
- Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand
| | - Chingching Foocharoen
- Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand.
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2
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Cheah JX, Perin J, Hughes M, Mecoli CA, Paik JJ, Hummers LK, Shah AA, McMahan ZH. Demographics and Clinical Features Associated with Abnormal Small Bowel Motility in Systemic Sclerosis. Rheumatology (Oxford) 2024:keae542. [PMID: 39374539 DOI: 10.1093/rheumatology/keae542] [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: 06/10/2024] [Revised: 09/03/2024] [Accepted: 09/14/2024] [Indexed: 10/09/2024] Open
Abstract
OBJECTIVE The small bowel is affected in up to 50% of systemic sclerosis (SSc) patients, and some patients experience severe complications. Our aim was to use specific statistical methods to compare demographic and clinical features of SSc patients with and without abnormal small bowel to better characterize patients at risk for this complication. METHODS SSc patients with gastrointestinal symptoms were prospectively enrolled and underwent a scintigraphy-based whole gut transit (WGT) study. A cross-sectional analysis was performed comparing clinical features between patients with and without abnormal small bowel transit by WGT. Univariate logistic regression models and multivariable models were used to examine the relationship between clinical features and abnormal small bowel transit. RESULTS Of 130 patients enrolled in this study, 22 had abnormal small bowel transit. SSc patients with abnormal small bowel transit were more likely to be male [Odds Ratio(OR)=3.70, Confidence Interval(CI) 1.07-12.50, p= 0.038], and have more severe cardiac involvement (OR = 3.98, CI 1.10-14.38, p= 0.035), while they were less likely to have sicca symptoms (OR = 0.30, CI 0.10-0.94, p= 0.039). In multivariable analyses, sicca symptoms (OR = 0.28, CI 0.08-0.96, p= 0.043) remained negatively associated with abnormal small bowel transit. Additionally, SSc patients with abnormal small bowel transit had higher mortality than patients with normal small bowel transit [Hazard ratio(HR)=4.57, CI 1.58-13.24, p= 0.005]. CONCLUSIONS These findings suggest that patients with abnormal small bowel transit in SSc are more likely to be male, have more severe cardiac involvement, higher mortality, and less sicca symptoms. Recognizing this patient subgroup is essential for risk stratification and optimizing clinical care.
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Affiliation(s)
- Jenice X Cheah
- Department of Medicine, University of California-Los Angeles, Los Angeles, CA
| | - Jamie Perin
- Department of International Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD
| | - Michael Hughes
- Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK
- Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK
| | - Christopher A Mecoli
- Johns Hopkins University, Department of Medicine, Division of Rheumatology, Baltimore, MD
| | - Julie J Paik
- Johns Hopkins University, Department of Medicine, Division of Rheumatology, Baltimore, MD
| | - Laura K Hummers
- Johns Hopkins University, Department of Medicine, Division of Rheumatology, Baltimore, MD
| | - Ami A Shah
- Johns Hopkins University, Department of Medicine, Division of Rheumatology, Baltimore, MD
| | - Zsuzsanna H McMahan
- Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX
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3
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Neto M, Albuquerque F, Oliveira J, Cadório MJ, Salvador MJ, Santiago T. Efficacy assessment of intravenous immunoglobulin for gastrointestinal involvement in systemic sclerosis using UCLA SCTC GIT: Case-based review. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2024:23971983241273852. [PMID: 39544902 PMCID: PMC11559525 DOI: 10.1177/23971983241273852] [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: 05/22/2024] [Accepted: 07/17/2024] [Indexed: 11/17/2024]
Abstract
Objectives To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department. Methods A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently. Results Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration. Conclusion The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field.
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Affiliation(s)
- Marcelo Neto
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
| | - Fernando Albuquerque
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
| | - João Oliveira
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
| | - Maria João Cadório
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
| | - Maria João Salvador
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
| | - Tânia Santiago
- Rheumatology Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal
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Quinlivan A, McMahan ZH, Lee EB, Nikpour M. Gastrointestinal Tract Considerations: Part II: How Should a Rheumatologist Best Manage Common Lower Gastrointestinal Tract Complaints in Systemic Sclerosis? Rheum Dis Clin North Am 2023; 49:319-336. [PMID: 37028837 DOI: 10.1016/j.rdc.2023.01.007] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/09/2023]
Abstract
Lower gastrointestinal (GI) symptoms are a frequently encountered problem for clinicians managing patients with systemic sclerosis. The current management practices are focused on the treatment of symptoms with little information available on how to use GI investigations in daily practice. This review demonstrates how to integrate the objective assessment of common lower GI symptoms into clinical care with the aim of guiding clinical decision making. Understanding the type of abnormal GI function that is affecting a patient and determining which parts of the gut are impacted can help clinicians to target therapy more precisely.
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Affiliation(s)
- Alannah Quinlivan
- Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, Victoria, 3065, Australia; Department of Medicine, The University of Melbourne at St Vincent's Hospital, 41 Victoria Parade, Fitzroy, Victoria 3065, Australia
| | - Zsuzsanna H McMahan
- Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Suite 5200, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, USA
| | - Eun Bong Lee
- Division of Rheumatology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul, Republic of Korea
| | - Mandana Nikpour
- Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, Victoria, 3065, Australia; Department of Medicine, The University of Melbourne at St Vincent's Hospital, 41 Victoria Parade, Fitzroy, Victoria 3065, Australia.
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van Leeuwen NM, Boonstra M, Fretheim H, Brunborg C, Midtvedt Ø, Garen T, Molberg Ø, Huizinga TWJ, de Vries-Bouwstra JK, Hoffman-Vold AM. Gastrointestinal symptom severity and progression in systemic sclerosis. Rheumatology (Oxford) 2022; 61:4024-4034. [PMID: 35238377 PMCID: PMC9789747 DOI: 10.1093/rheumatology/keac118] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/24/2020] [Revised: 02/17/2021] [Indexed: 01/01/2023] Open
Abstract
OBJECTIVES To evaluate the severity and evolution of patient-reported gastrointestinal tract (GIT) symptoms in systemic sclerosis (SSc) patients, assess predictive factors for progression and determine the impact of standard of care treatment. METHODS SSc patients from the Leiden and Oslo cohorts were included. We assessed clinical data and patient-reported GIT symptoms measured by the validated University of California, Los-Angeles Gastrointestinal-tract (UCLA-GIT) score at baseline and annually. GIT severity and progression was determined. Logistic regression was applied to identify risk factors associated with baseline GIT symptom severity. Linear mixed-effect models were applied to assess progression in GIT symptom burden and to identify predictive factors. We repeated all analysis in patients with early disease (inception cohort) to exclude the effect of longstanding disease and increase insights in development of GIT symptom burden early in the disease course. RESULTS We included 834 SSc patients with baseline UCLA GIT scores, 454 from Leiden and 380 from Oslo. In the total cohort, 28% reported moderate-severe GIT symptoms at baseline, with increased risk for severity conferred by ACA, smoking and corticosteroid use, while use of calcium channel blockers appeared protective. In the inception cohort, 23% reported moderate-severe GIT symptoms at baseline, with increased risk for females and with smoking. Over time, symptom burden increased mainly for reflux/bloating. Female sex and ACA predicted GIT symptom progression. CONCLUSION High GIT symptom burden is present early in SSc disease course. Both for prevalence and for progression of GIT symptom burden, female sex and smoking were identified as risk factors.
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Affiliation(s)
- Nina M van Leeuwen
- Correspondence to: Nina Marijn van Leeuwen, Department of Rheumatology, Leiden University Medical Center, Albinusdreef 2, 4333ZA, Leiden, The Netherlands. E-mail:
| | - Maaike Boonstra
- Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
| | | | - Cathrine Brunborg
- Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital
| | | | | | - Øyvind Molberg
- Department of Rheumatology,Institute of Clinical Medicine, University of Oslo, Oslo, Norway
| | - Tom W J Huizinga
- Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
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Mugo GW, Murunga EM. Systemic sclerosis manifesting as intractable gastro-oesophageal reflux and diarrhoea: a case report from Kenya. Pan Afr Med J 2021; 39:225. [PMID: 34630837 PMCID: PMC8486939 DOI: 10.11604/pamj.2021.39.225.29771] [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: 05/10/2021] [Accepted: 07/15/2021] [Indexed: 11/11/2022] Open
Abstract
Systemic sclerosis is a rare condition that has not been well reported in Africa, and several multisystemic manifestations, including gastrointestinal ones, have not been well documented locally. We present an unusual case of persistent gastro-oesophageal reflux and diarrhoea in a 74-year-old Kenyan female, who progressively developed abdominal distention, dysphagia and Raynaud´s phenomenon. Stool tests were unremarkable, whereas antinuclear antibody, ribonucleoproteins antibody (anti-nRNP/Sm) and anti-Sjögren's-syndrome-related antigen A autoantibody (anti-SSA) tests were positive. Endoscopic and imaging investigations revealed features of gastrointestinal dysmotility including reflux oesophagitis, gastroparesis and chronic intestinal pseudo-obstruction. A diagnosis of systemic sclerosis was made, and she responded well to medical treatment. We present this case to contribute to the limited literature of a disease associated with high morbidity and mortality, as well as encourage fellow clinicians to have a high level of suspicion in their differentials of persistent gastrointestinal dysmotility.
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Luquez-Mindiola A, Atuesta AJ, Gómez-Aldana AJ. Gastrointestinal manifestations of systemic sclerosis: An updated review. World J Clin Cases 2021; 9:6201-6217. [PMID: 34434988 PMCID: PMC8362561 DOI: 10.12998/wjcc.v9.i22.6201] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2021] [Revised: 03/30/2021] [Accepted: 06/07/2021] [Indexed: 02/06/2023] Open
Abstract
Systemic sclerosis is an autoimmune disease characterized by vascular disease, fibrosis of the skin, and internal organ dysfunction. Gastrointestinal involvement is the most frequent complication of internal organs, impacting up to 90% of patients. Gastrointestinal involvement can affect any region of the gastrointestinal tract from the mouth to the anus, with a predominance of disorders being observed at the level of the upper digestive tract. The gastrointestinal involvement primarily involves the esophagus, small bowel, and rectum. The severity of gastrointestinal involvement affects quality of life and is a marker of worse prognosis and mortality in these patients. In this review, we describe the current findings regarding gastrointestinal involvement by this entity.
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Affiliation(s)
| | - Alexis Javier Atuesta
- Department of Internal Medicine, Universidad Nacional de Colombia, Bogota 11711, Colombia
| | - Andres Jose Gómez-Aldana
- Department of Endoscopy, Santa Fe Foundation of Bogotá (Fundación Santa Fe de Bogotá), Bogotá 11711, Colombia
- Faculty of Medicine, Universidad de los Andes, Bogota 11711, Colombia
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Paolino S, Pacini G, Schenone C, Patanè M, Sulli A, Sukkar SG, Lercara A, Pizzorni C, Gotelli E, Cattelan F, Goegan F, Smith V, Cutolo M. Nutritional Status and Bone Microarchitecture in a Cohort of Systemic Sclerosis Patients. Nutrients 2020; 12:nu12061632. [PMID: 32492873 PMCID: PMC7353037 DOI: 10.3390/nu12061632] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Revised: 05/15/2020] [Accepted: 05/27/2020] [Indexed: 12/20/2022] Open
Abstract
Systemic sclerosis (SSc) is a connective tissue disease characterized by initial microvascular damage, immune system activation and progressive fibrosis with insufficiency of internal organs. Gastrointestinal (GI) involvement is characterized by atrophy of the smooth muscle and small bowel hypomotility, mainly resulting from an autonomic nerve dysfunction. These modifications significantly affect gut transit and nutrient absorption, thus leading to malnutrition deficit induced by malabsorption. Nutritional deficit induced by malabsorption might also lead to bone alterations. This study aims to evaluate the relationship between malnutrition and bone status. Thirty-six postmenopausal female patients fulfilling the ACR 2013 criteria for SSc underwent dual-energy X-ray absorptiometry scan (DXA) to detect quantitative lumbar spine bone mineral density (BMD) and trabecular bone score (TBS) analysis to detect bone quality. Data from DXA also allow to assess body composition and provide several quantitative parameters, including free fat mass index (FFMI) that identifies the patient with malnutrition (values <15 kg/m2 in women and 17 kg/m2 in men), according to the ESPEN criteria. Body mass index (BMI) was calculated for all SSc patients and every patient completed a diary reporting GI symptoms. Two groups of SSc patients with or without diagnosed malnutrition according to FFMI parameter were identified. Malnourished SSc patients showed significantly lower weight (p = 0.01) and BMI (p = 0.001), as well as lower serum levels of hemoglobin (p = 0.009), albumin (p = 0.002), PTH (p = 0.02) and 25OH-vitamin D (p = 0.008). DXA analysis showed significantly lower lumbar L1-L4 T-score (p = 0.009) and BMD values (p = 0.029) in malnourished SSc patients. Consistently, TBS values were significantly lower in malnourished patients (p = 0.008) and correlated with BMD (at any site) and serum albumin levels (p = 0.02). In addition, FFMI positively correlated with bone parameters as well as with symptoms of intestinal impairment in malnourished SSc patients. Finally, GI symptoms significantly correlated with BMD but not with TBS. This pilot study shows that in malnourished SSc patients (2015 ESPEN criteria: FFMI<15 kg/m2), an altered bone status significantly correlates with GI involvement, in terms of symptoms being mainly due to intestinal involvement together with the presence of selected serum biomarkers of malnutrition.
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Affiliation(s)
- Sabrina Paolino
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Greta Pacini
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Carlotta Schenone
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Massimo Patanè
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Alberto Sulli
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | | | - Adriano Lercara
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Carmen Pizzorni
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Emanuele Gotelli
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Francesco Cattelan
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Federica Goegan
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
| | - Vanessa Smith
- Department of Internal Medicine, Ghent University, St. Pietersnieuwstraat 33, 9000 Gent, Belgium
- Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Gent, Belgium
- Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), 9000 Ghent, Belgium
- Correspondence: (V.S.); (M.C.); Tel.: +39-335233621 (M.C.)
| | - Maurizio Cutolo
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, 16126 Genoa, Italy; (S.P.); (G.P.); (C.S.); (M.P.); (A.S.); (A.L.); (C.P.); (E.G.); (F.C.); (F.G.)
- Correspondence: (V.S.); (M.C.); Tel.: +39-335233621 (M.C.)
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Arif T, Adil M, Singh Sodhi J, Hassan I. Assessment of modified Rodnan skin score and esophageal manometry in systemic sclerosis: a study correlating severity of skin and esophageal involvement by objective measures. ACTA DERMATOVENEROLOGICA ALPINA PANNONICA ET ADRIATICA 2018. [DOI: 10.15570/actaapa.2018.36] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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10
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Menys A, Saliakellis E, Borrelli O, Thapar N, Taylor SA, Watson T. The evolution of magnetic resonance enterography in the assessment of motility disorders in children. Eur J Radiol 2018; 107:105-110. [PMID: 30292253 DOI: 10.1016/j.ejrad.2018.08.017] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2018] [Revised: 08/02/2018] [Accepted: 08/20/2018] [Indexed: 12/11/2022]
Abstract
Gastrointestinal symptoms including constipation, diarrhoea, pain and bloating represent some of the most common clinical problems for patients. These symptoms can often be managed with cheap, widely available medication or will spontaneously resolve. However, for many patients, chronic GI symptoms persist and frequently come to dominate their lives. At one end of the spectrum there is Inflammatory Bowel Disease (IBD) with a clearly defined but expensive treatment pathway. Contrasting with this is Irritable Bowel Syndrome (IBS), likely a collection of pathologies, has a poorly standardised pathway with unsatisfactory clinical outcomes. Managing GI symptoms in adult populations is a challenge. The clinical burden of gastrointestinal disease is also prevalent in paediatric populations and perhaps even harder to treat. In this review we explore some of the recent advances in magnetic resonance imaging (MRI) to study the gastrointestinal tract. Complex in both its anatomical structure and its physiology we are likely missing key physiological markers of disease through relying on symptomatic descriptors of gut function. Using MRI we might be able to characterise previously opaque processes, such as non-propulsive contractility, that could lead to changes in how we understand even common symptoms like constipation. This review explores recent advances in the field in adult populations and examines how this safe, objective and increasingly available modality might be applied to paediatric populations.
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Affiliation(s)
- A Menys
- Centre for Medical Imaging, UCL, London, UK.
| | | | - O Borrelli
- Great Ormond Street Hospital, London, UK
| | - N Thapar
- Great Ormond Street Hospital, London, UK
| | - S A Taylor
- Centre for Medical Imaging, UCL, London, UK
| | - T Watson
- Great Ormond Street Hospital, London, UK
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11
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Asano Y, Jinnin M, Kawaguchi Y, Kuwana M, Goto D, Sato S, Takehara K, Hatano M, Fujimoto M, Mugii N, Ihn H. Diagnostic criteria, severity classification and guidelines of systemic sclerosis. J Dermatol 2018; 45:633-691. [PMID: 29687465 DOI: 10.1111/1346-8138.14162] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Accepted: 11/06/2017] [Indexed: 01/17/2023]
Abstract
Several effective drugs have been identified for the treatment of systemic sclerosis (SSc). However, in advanced cases, not only their effectiveness is reduced but they may be also harmful due to their side-effects. Therefore, early diagnosis and early treatment is most important for the treatment of SSc. We established diagnostic criteria for SSc in 2003 and early diagnostic criteria for SSc in 2011, for the purpose of developing evaluation of each organ in SSc. Moreover, in November 2013, the American College of Rheumatology and the European Rheumatology Association jointly developed new diagnostic criteria for increasing their sensitivity and specificity, so we revised our diagnostic criteria and severity classification of SSc. Furthermore, we have revised the clinical guideline based on the newest evidence. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of SSc.
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Affiliation(s)
- Yoshihide Asano
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masatoshi Jinnin
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Yasushi Kawaguchi
- Institute of Rheumatology, Tokyo Woman's Medical University, Tokyo, Japan
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
| | - Daisuke Goto
- Department of Rheumatology, Faculty of Medicine, Univertity of Tsukuba, Ibaraki, Japan
| | - Shinichi Sato
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kazuhiko Takehara
- Department of Molecular Pathology of Skin, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Ishikawa, Japan
| | - Masaru Hatano
- Graduate School of Medicine Department of Therapeutic Strategy for Heart Failure, The University of Tokyo, Tokyo, Japan
| | - Manabu Fujimoto
- Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Naoki Mugii
- Section of Rehabilitation, Kanazawa University Hospital, Ishikawa, Japan
| | - Hironobu Ihn
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
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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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13
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Malnutrition and sarcopenia in a large cohort of patients with systemic sclerosis. Clin Rheumatol 2017; 37:987-997. [PMID: 29196890 DOI: 10.1007/s10067-017-3932-y] [Citation(s) in RCA: 60] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2017] [Revised: 11/20/2017] [Accepted: 11/24/2017] [Indexed: 12/13/2022]
Abstract
Systemic sclerosis (SSc) is an autoimmune disease which may lead to malnutrition. Previous studies have defined it with different criteria. No thorough evaluations of sarcopenia in SSc are available. The aim of the present study was to assess the prevalence and the potential association of malnutrition and sarcopenia in a large cohort of SSc cases. A total of 141 SSc consecutive outpatients were enrolled. Body composition was analyzed by densitometry. Malnutrition was defined according to recently published ESPEN criteria, whereas sarcopenia was diagnosed in patients with reduced skeletal muscle index. Malnutrition was diagnosed in 9.2% of patients (95% CI, 4.4-14.0%). Malnourished patients had worse gastrointestinal symptoms according to UCLA SCTC GIT 2.0 questionnaire (p = 0.007), lower physical activity (p = 0.028), longer disease duration (p = 0.019), worse predicted DLCO/VA and FVC (p = 0.009, respectively), worse disease severity according to Medsger severity score (p < 0.001), lower hemoglobin (p = 0.023), and fat-free mass (p < 0.001) and were more often sarcopenic (p < 0.001). In multivariate analysis, only FVC (p = 0.006) and disease severity (p = 0.003), in particular for the lungs (p = 0.013), were confirmed to be worse in malnourished patients. Sarcopenia was diagnosed in 29\140 patients (20.7%; 95% CI, 14.0-27.4%); 11\29 were also malnourished. In multivariate analysis, sarcopenic patients had longer disease duration (p = 0.049), worse DLCO/VA (p = 0.002), and lung (p = 0.006) and skin (p = 0.014) involvement. In SSc, malnutrition defined with ESPEN criteria was found to be lower than previously reported. Sarcopenia was found to be somewhat common. Lung involvement was significantly associated with nutritional status and may not be explained only by muscle weakness.
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The Microbiome in Connective Tissue Diseases and Vasculitides: An Updated Narrative Review. J Immunol Res 2017; 2017:6836498. [PMID: 28835902 PMCID: PMC5556609 DOI: 10.1155/2017/6836498] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2017] [Revised: 07/04/2017] [Accepted: 07/12/2017] [Indexed: 02/07/2023] Open
Abstract
Objective To provide a narrative review of the most recent data concerning the involvement of the microbiome in the pathogenesis of connective tissue diseases (CTDs) and vasculitides. Methods The PubMed database was searched for articles using combinations of words or terms that included systemic lupus erythematosus, systemic sclerosis, autoimmune myositis, Sjögren's syndrome, undifferentiated and mixed CTD, vasculitis, microbiota, microbiome, and dysbiosis. Papers from the reference lists of the articles and book chapters were reviewed, and relevant publications were identified. Abstracts and articles written in languages other than English were excluded. Results We found some evidence that dysbiosis participates in the pathogenesis of systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, and Behçet's disease, but there are still few data concerning the role of dysbiosis in other CTDs or vasculitides. Conclusions Numerous studies suggest that alterations in human microbiota may be involved in the pathogenesis of inflammatory arthritides as a result of the aberrant activation of the innate and adaptive immune responses. Only a few studies have explored the involvement of dysbiosis in other CTDs or vasculitides, and further research is needed.
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Abstract
Systemic sclerosis (SSc) is an autoimmune connective tissue disorder. Anorectal involvement might typically cause fecal incontinence and rarely rectal prolapse. Here we report three female patients, who were admitted with a mean history of 10 years suffering from SSc. All patients presented with the initial symptom of anal incontinence, in all cases this was associated with rectal intussusception or rectal prolapse. The three women faced prolapse recurrence, independent of the initial procedure. After surgical removal of the prolapse, the incontinence remained. In SSc rectal prolapse syndrome might occur at an earlier age, and a primary prolapse of the ventral aspect of the rectal wall seems to be typical for this disease. If patients with prior diagnosis of SSc appear with third degree of fecal incontinence, it is suspected to be associated with rectal prolapse. The prolapse recurrence rate after surgery in SSc patients is high.
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Association between Clinical Manifestations of Systemic Sclerosis and Esophageal Dysmotility Assessed by High-Resolution Manometry. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2017. [DOI: 10.5301/jsrd.5000233] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Purpose To characterize esophageal involvement according to high-resolution manometry (HRM) findings using the 3rd version of the Chicago Classification, in a French population of patients fulfilling the ACR/EULAR 2013 classification criteria for systemic sclerosis (SSc). Methods Thirty-six patients were consecutively included in this cross-sectional non-interventional study and had HRM performed in Rennes University hospital. Demographic and clinical characteristics, SSc history and interstitial lung disease (ILD) on CT-scan were assessed, and compared with esophageal motility. Results Sixty-one percent of SSc patients had ineffective peristalsis (55.6% failed peristalsis and 5.6% weak peristalsis), 33.3% had hypotensive esophagogastric junction pressure, 75% did not have a physiologic contraction following multiple rapid swallow (MRS), and 44.4% had an abnormal peristaltic reserve. One patient had type 1 achalasia and another one had Jackhammer esophagus. Failed peristalsis was associated with pyrosis (odds ratio [OR] 7.28, 95% confidence interval [CI] 1.51-35.21, p = 0.009), a higher modified Rodnan skin score (MRSS) (without failed peristalsis: 4.68 ± 2.95 vs. with failed peristalsis: 10.68 ± 9.23; p<0.05), the presence of telangiectasia (OR 7, 95% CI 1.59-30.8, p = 0.007), and low diffusing capacity of the lung for carbon monoxide (DLCO) (p = 0.013). Food in the esophagus and esophageal dilation on CT-scan were associated with failed contractions on HRM (respectively, OR 6.85, 95% CI 1.12-40.82, p = 0.05, and OR 14.67, CI 2.4-88.5, p = 0.002). Conclusions This study confirms that failed peristalsis is frequent in SSc and associated with other organ involvement. We found a concordance between HRM results and CT-scan findings regarding esophageal involvement.
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Krogh K, Chiarioni G, Whitehead W. Management of chronic constipation in adults. United European Gastroenterol J 2016; 5:465-472. [PMID: 28588875 DOI: 10.1177/2050640616663439] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2016] [Accepted: 07/15/2016] [Indexed: 12/21/2022] Open
Abstract
Chronic constipation (CC) is an extremely common condition with an estimated prevalence of up to 24%. Most patients with CC should be treated in primary care. Changes in lifestyle, including increased intake of dietary fibre, fluid, and exercise, should be attempted in most patients. Osmotic or stimulant laxatives are indicated if there are insufficient effects of lifestyle changes. Prokinetics and secretagogues should be restricted to those not responding to basic treatment. Anorectal physiology tests and assessment of colorectal transit time are indicated if medical treatment fails or if symptoms indicate severely obstructed defecation. If symptoms indicate an underlying structural disorder, barium or magnetic resonance evacuation proctography is indicated. Biofeedback therapy is effective in patients with dyssynergic defecation. In patients with other evacuation disorders, rectally administered laxatives or transanal irrigation should be attempted. Surgery is restricted to the minority of CC patients with very severe symptoms not responding to conservative treatment.
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Affiliation(s)
- K Krogh
- Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark
| | - G Chiarioni
- Gastroenterology Division, University of Verona, Verona, Italy
| | - W Whitehead
- Division of Gastroenterology and Hepatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.,Center for Functional Gastrointestinal and Motility Disorders, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
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18
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Emmanuel A. Current management of the gastrointestinal complications of systemic sclerosis. Nat Rev Gastroenterol Hepatol 2016; 13:461-72. [PMID: 27381075 DOI: 10.1038/nrgastro.2016.99] [Citation(s) in RCA: 50] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Systemic sclerosis is a multisystem autoimmune disorder that involves the gastrointestinal tract in more than 90% of patients. This involvement can extend from the mouth to the anus, with the oesophagus and anorectum most frequently affected. Gut complications result in a plethora of presentations that impair oral intake and faecal continence and, consequently, have an adverse effect on patient quality of life, resulting in referral to gastroenterologists. The cornerstones of gastrointestinal symptom management are to optimize symptom relief and monitor for complications, in particular anaemia and malabsorption. Early intervention in patients who develop these complications is critical to minimize disease progression and improve prognosis. In the future, enhanced therapeutic strategies should be developed, based on an ever-improving understanding of the intestinal pathophysiology of systemic sclerosis. This Review describes the most commonly occurring clinical scenarios of gastrointestinal involvement in patients with systemic sclerosis as they present to the gastroenterologist, with recommendations for the suggested assessment protocol and therapy in each situation.
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Affiliation(s)
- Anton Emmanuel
- Gastrointestinal Physiology Unit, University College Hospital, 235 Euston Road, London NW1 2BU, UK
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Menys A, Butt S, Emmanuel A, Plumb AA, Fikree A, Knowles C, Atkinson D, Zarate N, Halligan S, Taylor SA. Comparative quantitative assessment of global small bowel motility using magnetic resonance imaging in chronic intestinal pseudo-obstruction and healthy controls. Neurogastroenterol Motil 2016; 28:376-83. [PMID: 26661570 DOI: 10.1111/nmo.12735] [Citation(s) in RCA: 42] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2015] [Accepted: 10/30/2015] [Indexed: 12/12/2022]
Abstract
BACKGROUND Chronic intestinal pseudo-obstruction (CIPO) is characterized by dilatation of the bowel lumen and abnormal motility. In this study, we aimed to quantify small bowel dysmotility in CIPO using a validated pan-intestinal motility assessment technique based on motion capture magnetic resonance imaging (MRI) compared to normal controls. In addition, we explored if motility responses of CIPO patients to neostigmine challenge differed from healthy volunteers. METHODS Twenty healthy volunteers (mean age 28, range 22-48) and 11 CIPO patients (mean age 47, range 19-90) underwent MRI enterography to capture global small bowel motility. Eleven controls and seven CIPO patients further underwent a randomized placebo-controlled crossover study of either intravenous neostigmine (0.5 mg) or saline with motility MRI repeated at a mean of 3 weeks. Motility was quantified in regions of interest placed to encompass the whole small bowel volume using a validated, postprocessing technique to give a global motility index in arbitrary units (AU). Baseline and stimulated motility was compared using Wilcoxon rank-sum paired T-tests. KEY RESULTS Baseline global small bowel motility was significantly lower in CIPO patients compared to controls (mean 0.25 AU vs 0.35 AU, p < 0.001). Motility in both groups increased significantly after neostigmine (0.06 AU increase, p = 0.016 in CIPO and 0.06 AU increase, p = 0.002 in controls). Three patients with scleroderma had a reduced response to neostigmine. CONCLUSIONS & INFERENCES Global small bowel motility in CIPO patients is significantly lower than controls and response to the pro-kinetic agent neostigmine may differ according to disease phenotype. Software-quantified bowel motility using cine MRI has potential as a future tool to investigate enteric dysmotility.
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Affiliation(s)
- A Menys
- Centre for Medical Imaging, UCL, London, UK
| | - S Butt
- Gastroenterology, University College London Hospitals, London, UK
| | - A Emmanuel
- Gastroenterology, University College London Hospitals, London, UK
| | - A A Plumb
- Centre for Medical Imaging, UCL, London, UK
| | - A Fikree
- Wingate Institute of Neurogastroenterology, Centre for Digestive Diseases, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University, London, UK
| | - C Knowles
- Wingate Institute of Neurogastroenterology, Centre for Digestive Diseases, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, Queen Mary University, London, UK
| | - D Atkinson
- Centre for Medical Imaging, UCL, London, UK
| | - N Zarate
- Gastroenterology, University College London Hospitals, London, UK
| | - S Halligan
- Centre for Medical Imaging, UCL, London, UK
| | - S A Taylor
- Centre for Medical Imaging, UCL, London, UK
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Oxalate nephropathy in systemic sclerosis: Case series and review of the literature. Semin Arthritis Rheum 2015; 45:315-20. [PMID: 26239907 DOI: 10.1016/j.semarthrit.2015.06.017] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2015] [Revised: 06/08/2015] [Accepted: 06/30/2015] [Indexed: 01/16/2023]
Abstract
OBJECTIVE To increase awareness of oxalate nephropathy as a cause of acute kidney injury (AKI) among systemic sclerosis patients with small intestinal dysmotility and malabsorption, and to prompt consideration of dietary modification and early treatment of predisposing causes of oxalate nephropathy in this population. METHODS Two cases of biopsy-proven oxalate nephropathy were identified among systemic sclerosis patients in the course of direct clinical care. Subsequently, a retrospective search of the Johns Hopkins Pathology databases identified a third patient with systemic sclerosis who developed oxalate nephropathy. RESULTS Among the three patients with qualifying biopsies, all three had systemic sclerosis with lower gastrointestinal involvement. All three presented with diarrhea, malabsorption, and AKI. In two of the three patients, diarrhea was present for at least 2 years before the development of AKI; in the third, incidental oxalate nephropathy was noted 3 years before she developed AKI and extensive oxalate nephropathy in the setting of a prolonged mycobacterium avium-intracellulare enteritis. In the first case, oxalate crystals were present by urinalysis months before diagnosis by biopsy; in the second, hyperoxaluria was diagnosed by urine collection immediately after; and in the third, oxalate crystals had been noted incidentally on post-transplant renal biopsy 3 years before the development of fulminant oxalate nephropathy. All three patients died within a year after diagnosis. CONCLUSIONS Patients with systemic sclerosis and bowel dysmotility associated with chronic diarrhea and malabsorption may be at risk for an associated oxalate nephropathy. Regular screening of systemic sclerosis patients with small bowel malabsorption syndromes through routine urinalysis or 24-h urine oxalate collection, should be considered. Further studies defining the prevalence of this complication in systemic sclerosis, the benefit of dietary modification on hyperoxaluria, the effect of treating small intestinal bowel overgrowth with antibiotics, and the effectiveness of probiotics, calcium supplements, or magnesium supplements to prevent hyperoxaluria-associated renal disease in these patients, are warranted.
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Abstract
PURPOSE OF REVIEW The gastrointestinal tract is the most common extra-cutaneous organ system involved with systemic sclerosis (SSc) affecting approximately 90% of patients. This review summarizes the recent advances in the evaluation and management of gastrointestinal manifestations of SSc. RECENT FINDINGS There is a growing body of evidence that uncontrolled GERD can play a significant role in the pathogenesis of SSc-associated interstitial lung disease. Newer forms of management of Barrett esophagus are showing significant promise as potentially curative therapy. Gastric antral vascular ectasias have strongly been associated with the presence of RNA polymerase III antibody. Newer technologies have advanced the assessment of gastrointestinal dysmotility in SSc. Evidence of probiotic use for the treatment of gastrointestinal complications is emerging. The UCLA SCTC GIT 2.0 questionnaire is being increasingly accepted by the SSc experts as a validated instrument for evaluation of patient-reported outcomes involving the gastrointestinal tract. SUMMARY Our knowledge of the complex pathogenesis of gastrointestinal manifestations of SSc has expanded substantially in the last few decades. There has also been considerable technological progress in the evaluation of these manifestations. Patient care is being optimized by close collaboration of rheumatologists and gastroenterologists, leading to a more coordinated approach in the management of these complications.
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Abstract
The gastrointestinal tract, affecting more than 90% of patients, is the internal organ most frequently involved in systemic sclerosis. Any part of the gastrointestinal tract can be affected, from the mouth to the anus. Patients often experience reduced quality of life and impaired social life. Although only 8% have severe gastrointestinal involvement, mortality is high in those patients. Recent studies on the pathophysiology of the disease highlight new mechanisms to explain gastrointestinal dysmotility, but treatment remains symptomatic. This article reviews the pathophysiology of the gastrointestinal tract and discusses the investigation and management of the disease.
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Affiliation(s)
- Genevieve Gyger
- Division of Rheumatology, Jewish General Hospital, McGill University, Suite A725, 3755 Cote St Catherine Road, Montreal, Quebec H3T 1E2, Canada.
| | - Murray Baron
- Division of Rheumatology, Jewish General Hospital, McGill University, Suite A725, 3755 Cote St Catherine Road, Montreal, Quebec H3T 1E2, Canada
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Nagaraja V, McMahan ZH, Getzug T, Khanna D. Management of gastrointestinal involvement in scleroderma. CURRENT TREATMENT OPTIONS IN RHEUMATOLOGY 2015; 1:82-105. [PMID: 26005632 DOI: 10.1007/s40674-014-0005-0] [Citation(s) in RCA: 57] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Gastrointestinal tract (GIT) commonly affects patients with systemic sclerosis (SSc). The GI involvement is quite heterogeneous varying from asymptomatic disease to significant dysmotility causing complications like malabsorption, weight loss and severe malnutrition. This review focuses on the management of GI involvement in SSc and has been categorized based on the segment of GIT involved. A brief discussion on the role of patient reported outcome measures in SSc-GI involvement has also been incorporated.
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Affiliation(s)
- Vivek Nagaraja
- Division of Rheumatology, University of Toledo, Toledo, Ohio
| | | | - Terri Getzug
- Division of Rheumatology, University of Toledo, Toledo, Ohio
| | - Dinesh Khanna
- Division of Rheumatology, University of Michigan, Ann Arbor, Michigan
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