Mayank Jha, Mianbo Wang, Russell Steele, Murray Baron, Marvin J Fritzler, Marie Hudson
{"title":"NT-proBNP, hs-cTnT, and CRP predict the risk of cardiopulmonary outcomes in systemic sclerosis: Findings from the Canadian Scleroderma Research Group.","authors":"Mayank Jha, Mianbo Wang, Russell Steele, Murray Baron, Marvin J Fritzler, Marie Hudson","doi":"10.1177/23971983211040608","DOIUrl":"https://doi.org/10.1177/23971983211040608","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to determine the independent value of N-terminal pro b-type natriuretic peptide, high-sensitivity cardiac troponin T, and C-reactive protein to predict onset of cardiopulmonary disease in a large, multi-center systemic sclerosis cohort followed prospectively.</p><p><strong>Methods: </strong>Subjects from the Canadian Scleroderma Research Group registry with data on N-terminal pro b-type natriuretic peptide, high-sensitivity cardiac troponin T, and C-reactive protein were identified. Outcomes of interest were death, systolic dysfunction (left ventricular ejection fraction < 50% or medications for heart failure), pulmonary arterial hypertension by right heart catheterization, pulmonary hypertension by cardiac echocardiography (systolic pulmonary artery pressures ⩾ 45 mmHg), arrhythmias (pacemaker/implantable cardiac defibrillator or anti-arrhythmic medications), and interstitial lung disease. Multivariate Cox proportional hazard models were generated for each outcome.</p><p><strong>Results: </strong>A total of 675 subjects were included with a mean follow-up of 3.0 ± 1.8 years. Subjects were predominantly women (88.4%) with mean age of 58.2 ± 11.3 years and mean disease duration of 13.7 ± 9.1 years. One hundred and one (101, 15%) subjects died during follow-up, 37 (6.4 %) developed systolic dysfunction, 18 (2.9%) arrhythmias, 34 (5.1%) pulmonary arterial hypertension, 43 (7.3%) pulmonary hypertension, and 48 (12.3%) interstitial lung disease. In multivariate analyses, elevated levels of N-terminal pro b-type natriuretic peptide, high-sensitivity cardiac troponin T, and C-reactive protein were associated with increased risk of death, while elevated levels of N-terminal pro b-type natriuretic peptide and C-reactive protein were associated with increased risk of developing pulmonary hypertension.</p><p><strong>Conclusion: </strong>In systemic sclerosis, N-terminal pro b-type natriuretic peptide, high-sensitivity cardiac troponin T, and C-reactive protein have independent predictive value for death and pulmonary hypertension. A larger study would be required to determine the predictive value of these biomarkers for less common systemic sclerosis outcomes.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8922674/pdf/10.1177_23971983211040608.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9149155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yannick Allanore, Joel Constans, Dominique Godard, Gerard de Pouvourville, Stephane Bouee, Viviane Jeanbat, Clement Teissier, Katell Le Lay, Julien Chollet, Eric Hachulla
{"title":"Quality of life in SSc-ILD patients: Understanding the impact of the ILD and the needs of the SSc-ILD patients and their need for caregivers in France.","authors":"Yannick Allanore, Joel Constans, Dominique Godard, Gerard de Pouvourville, Stephane Bouee, Viviane Jeanbat, Clement Teissier, Katell Le Lay, Julien Chollet, Eric Hachulla","doi":"10.1177/23971983211013979","DOIUrl":"10.1177/23971983211013979","url":null,"abstract":"<p><strong>Objectives: </strong>The objectives of this study were to describe the impact of systemic sclerosis associated interstitial lung disease, on quality of life, to estimate the correlation between quality of life and severity of lung disease and to assess the impact of interstitial lung disease on caregivers.</p><p><strong>Methods: </strong>Seven investigators included systemic sclerosis associated interstitial lung disease patients from December 2019 to April 2020. Sociodemographics and clinical data were collected. Patients reported outcomes and questionnaires were used with 1 generic patients reported outcome (EQ-5D-5L), 1 specific PRO (Brief Interstitial Lung Disease) and 2 self-reported questionnaires on impact of SSc complications and impact on caregivers. The correlation between forced vital capacity and EQ-5D-5L score was estimated with a multivariate linear regression model adjusted on several covariates.</p><p><strong>Results: </strong>In all, 89 patients were included. 26.4% were males, mean age was 58.2 ± 14.5 years. Mean EQ-5D-5L score = 0.79 ± 0.22 (median = 0.85). Mean EQ-5D-5L visual analog scale score = 60.8 ± 20.4 (median = 61.5). Mean King's Brief Interstitial Lung Disease score = 58.4 ± 12.7 (median = 58.0). After adjustment on covariates, a significant correlation between forced vital capacity and EQ-5D-5L score was found with an increase of 0.003 of the EQ-5D-5L score for a 1% increase of FVC (p = 0.0096). No significant correlation between forced vital capacity and the EQ-VAS and King's Brief Interstitial Lung Disease score were found. The impact of SSc on other organs was significantly correlated with EQ- 5D-5L score, respectively, for the impact scores on the lung system (p = 0.0003), heart system (p = 0.0182), Raynaud's syndrome (p = 0.0015), digestive system (p = 0.0032), joints/muscles (p = 0.0003), skin (p < 0.0001), kidney (p = 0.0052) and gastro-oesophageal reflux (p = 0.0063). Significant correlations between King's Brief Interstitial Lung Disease score and lung system (p < 0.0001), heart system (p < 0.0001), digital ulcers (p = 0.058), digestive system (p < 0.0001), kidney (p = 0.0004), skin (p = 0.0499) and gastro-oesophageal reflux (p = 0.0033) scores were found 68.5% of patients reported their need for a caregiver to help them in their daily life activities.</p><p><strong>Conclusion: </strong>Our study highlighted the strong burden of systemic sclerosis associated interstitial lung disease` for patients, especially with an impact on quality of life, on other organs manifestations and need for caregivers in their daily life.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8922678/pdf/10.1177_23971983211013979.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9149153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Scleroderma renal crisis following Covid-19 infection.","authors":"Doron Rimar, Itzhak Rosner, Gleb Slobodin","doi":"10.1177/23971983211016195","DOIUrl":"10.1177/23971983211016195","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is an autoimmune disease in which environmental exposure to substances and agents may trigger disease onset or exacerbation. The most fatal complication of SSc is scleroderma renal crisis (SRC), the incidence of which is 2-3%. SRC usually occurs in the first 5 years from disease onset in diffuse-SSc patients with anti-topoisomerase 1 (ATA) or RNA polymerase 3 antibodies [1]. Other risk factors for SRC are pericardial effusion, tendon friction rub and steroid use. We report herein a case of scleroderma renal crisis (SRC), following covid-19 infection, in a limited-SSc patient who was in long remission prior to the infection without any risk factors for SRC. the temporal relationship and lack of other risk factors combine to suggest covid-19 infection as a possible trigger for SRC. We discuss the shared pathophysiology of covid-19 infection and SRC, including, vasculopathy, endothelial activation, hypercoagulability, cytokines release as interleukin 6, that may explain the possible role of covid-19 infection, as a trigger for SRC in SSc patients.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8922670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46655434","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kristi Kong, Diane Koontz, Christina Morse, Eileen Roth, Robyn T Domsic, Marc A Simon, Eric Stratton, Connor Buchholz, Kimberly Tobin-Finch, Robert Simms, M Patricia George, Paul M Hassoun, Harrison Farber, Robert Lafyatis
{"title":"A Pilot Study of Dimethyl Fumarate in Pulmonary Arterial Hypertension Associated with Systemic Sclerosis.","authors":"Kristi Kong, Diane Koontz, Christina Morse, Eileen Roth, Robyn T Domsic, Marc A Simon, Eric Stratton, Connor Buchholz, Kimberly Tobin-Finch, Robert Simms, M Patricia George, Paul M Hassoun, Harrison Farber, Robert Lafyatis","doi":"10.1177/23971983211016196","DOIUrl":"https://doi.org/10.1177/23971983211016196","url":null,"abstract":"<p><strong>Introduction: </strong>Given the poor treatment options for pulmonary arterial hypertension associated systemic sclerosis (SSc-PAH) patients, we sought to determine clinical safety and efficacy of Dimethylfumarate (DMF), an Nrf2 agonist, and the effects on biomarkers of oxidative stress on SSc-PAH in an exploratory interventional clinical trial.</p><p><strong>Objectives: </strong>The primary objectives were to assess the safety and efficacy of treatment with DMF in patients with SSc-PAH.</p><p><strong>Methods: </strong>This was an investigator-initiated, double-blind, randomized, placebo-controlled trial conducted at two sites in the United States. The primary safety endpoint was the incidence of serious adverse events (SAEs) and all adverse events (AEs) in DMF compared to placebo-treated patients. The primary efficacy endpoint was the change in 6MWD from baseline to the end of treatment at Week 24 in DMF compared to placebo-treated patients.</p><p><strong>Results: </strong>Six participants were randomized to either placebo (n = 2) or DMF (n = 4). Baseline demographics were similar in both groups. A total of 25 adverse events (AEs) occurred in 6 subjects, with 14 AEs (56.0%) having occurred in DMF-treated subjects. 3 occurrences were identified as nausea AEs, and two participants withdrew due to nausea. One participant in the placebo group was withdrawn after a hospitalization SAE due to worsening of heart failure and shortness of breath secondary to anemia. One participant in each group completed protocol. Subjects in the DMF-treated group showed a non-significant reduced decline in 6MWD (relative mean change of -7.07%) from baseline to Week 24 as compared to placebo-treated subjects (relative mean change of -14.97%).</p><p><strong>Conclusion: </strong>Patients treated for SSc-PAH with 2 and 3-drug regimens, as is now typical for these patients, tolerate DMF poorly. Our small samples size did not provide power to suggest efficacy. We suggest that Nrf2 is still a valid therapeutic target for future trials, using better tolerated Nrf2 agonists.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/23971983211016196","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39663855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Scleroderma as paraneoplastic disease: A new case of a rare association with renal cell carcinoma.","authors":"Francesca Ruffilli, Melissa Padovan, Giovanni Ciancio, Veronica Venturelli, Beatrice Maranini, Marcello Govoni","doi":"10.1177/23971983211050810","DOIUrl":"10.1177/23971983211050810","url":null,"abstract":"","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8922656/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48811109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The role of interleukin-17 in the pathogenesis of systemic sclerosis: Pro-fibrotic or anti-fibrotic?","authors":"S. Bellando-Randone, E. Della-Torre, A. Balanescu","doi":"10.1177/23971983211039421","DOIUrl":"https://doi.org/10.1177/23971983211039421","url":null,"abstract":"Systemic sclerosis is characterized by widespread fibrosis of the skin and internal organs, vascular impairment, and dysregulation of innate and adaptive immune system. Growing evidence indicates that T-cell proliferation and cytokine secretion play a major role in the initiation of systemic sclerosis, but the role of T helper 17 cells and of interleukin-17 cytokines in the development and progression of the disease remains controversial. In particular, an equally distributed body of literature supports both pro-fibrotic and anti-fibrotic effects of interleukin-17, suggesting a complex and nuanced role of this cytokine in systemic sclerosis pathogenesis that may vary depending on disease stage, target cells in affected organs, and inflammatory milieu. Although interleukin-17 already represents an established therapeutic target for several immune-mediated inflammatory diseases, more robust experimental evidence is required to clarify whether it may become an attractive therapeutic target for systemic sclerosis as well.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43331602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Circulating Gremlin-1 is elevated in systemic sclerosis patients","authors":"S. O’Reilly","doi":"10.1177/23971983211036571","DOIUrl":"https://doi.org/10.1177/23971983211036571","url":null,"abstract":"Introduction: Systemic sclerosis is an autoimmune connective tissue disease in which there is activation of the immune system, vascular disease and fibrosis. Activation of quiescent fibroblasts to myofibroblasts is key to disease pathogenesis. Gremlin-1 is a bone morphogenetic protein antagonist which is important in development and we recently reported in skin fibrosis. The aim of this study was to determine the serum circulating levels of Gremlin-1 in early diffuse systemic sclerosis. Methods: Twenty-one early diffuse systemic sclerosis patients (less than 2 years from first non-Raynaud’s symptom) were included and age and sex-matched healthy controls. Serum was isolated from blood and measured with a specific enzyme-linked immunoassay for Gremlin-1. Clinical variables were also measured. Results: Significantly elevated Gremlin-1 was found in sera of early diffuse systemic sclerosis patients (p < 0.001). In patients with interstitial lung disease, this compared to systemic sclerosis without evidence of interstitial lung disease, Gremlin-1 was significantly elevated (p < 0.0007). A correlation was found between circulating Gremlin-1 and modified Rodnan Skin Score, albeit weak. Discussion: In early diffuse systemic sclerosis patients, elevated Gremlin-1 is found in serum. This is particularly prominent in systemic sclerosis–associated interstitial lung disease. This suggests that Gremlin-1 may be a biomarker for systemic sclerosis interstitial lung disease.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/23971983211036571","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45389696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
David P DeMasters, Emily L. Sturgill, A. Bartholomew
{"title":"Is axillary botulinum toxin efficient in controlling secondary Raynaud’s phenomenon? A case report","authors":"David P DeMasters, Emily L. Sturgill, A. Bartholomew","doi":"10.1177/23971983211034077","DOIUrl":"https://doi.org/10.1177/23971983211034077","url":null,"abstract":"Raynaud’s phenomenon when secondary to underlying systemic disease such as systemic sclerosis occurs early in the disease course and progression can bring significant morbidity such as pain, digital ulceration, and necrosis. Standard medical therapies are aimed at promoting distal arterial vasodilation but are often inadequate in managing Raynaud’s phenomenon. Options for refractory cases include surgical and chemical sympathectomy with Botulinum neurotoxin type A (BoNT/A) hand injections but the latter can be associated with transient hand weakness. We describe the case of a 35-year-old woman with undifferentiated connective tissue disease, Raynaud’s phenomenon, and concomitant primary focal axillary hyperhidrosis for which she received axillary BoNT/A therapy every 6 months who noted significant improvement in her Raynaud’s phenomenon and hand arthralgias for 5 months following the axillary injections. This effect remained durable after 24 months of therapy. This improvement in Raynaud’s phenomenon after axillary BoNT/A has not been previously described.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/23971983211034077","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49116744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Y. Radwan, Reto D. Kurmann, A. Sandhu, E. El-Am, C. Crowson, E. Matteson, T. Osborn, K. Warrington, R. Mankad, A. Makol
{"title":"Systemic sclerosis portends increased risk of conduction and rhythm abnormalities at diagnosis and during disease course: A US population-based cohort","authors":"Y. Radwan, Reto D. Kurmann, A. Sandhu, E. El-Am, C. Crowson, E. Matteson, T. Osborn, K. Warrington, R. Mankad, A. Makol","doi":"10.1177/23971983211034074","DOIUrl":"https://doi.org/10.1177/23971983211034074","url":null,"abstract":"Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% (p = 0.06), and any rhythm disorder was 18% versus 13% (p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/23971983211034074","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44490879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniel Levin, G. De Palma, Hannah Zou, A. Bazzaz, E. Verdú, Barbara Baker, M. Pinto-Sanchez, N. Khalidi, M. Larché, K. Beattie, P. Bercik
{"title":"Fecal microbiome differs between patients with systemic sclerosis with and without small intestinal bacterial overgrowth","authors":"Daniel Levin, G. De Palma, Hannah Zou, A. Bazzaz, E. Verdú, Barbara Baker, M. Pinto-Sanchez, N. Khalidi, M. Larché, K. Beattie, P. Bercik","doi":"10.1177/23971983211032808","DOIUrl":"https://doi.org/10.1177/23971983211032808","url":null,"abstract":"Introduction: Gastrointestinal manifestations of systemic sclerosis affect up to 90% of patients, with symptoms including diarrhea and constipation. Small intestinal bacterial overgrowth is a condition associated with increased numbers of pathogenic bacteria in the small bowel. While currently unknown, it has been suggested that dysregulation of the fecal microbiota may play a role in the development of systemic sclerosis and small intestinal bacterial overgrowth. Objectives: Our study aimed to describe the fecal microbiota of patients with systemic sclerosis and compare it between those with and without a diagnosis of small intestinal bacterial overgrowth. We also compared the fecal microbiota of systemic sclerosis patients with that of healthy controls to understand the association between particular bacterial taxa and clinical gastrointestinal manifestations of systemic sclerosis. Methods: A total of 29 patients with systemic sclerosis underwent breath testing to assess for small intestinal bacterial overgrowth, provided stool samples to determine taxonomic assignments, and completed the University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0, which details symptoms and quality-of-life factors. Stool samples were compared between systemic sclerosis patients with and without small intestinal bacterial overgrowth, and between patients with systemic sclerosis and a healthy control cohort (n = 20), aged 18–80 years. Results: Fecal microbiome analyses demonstrated differences between systemic sclerosis patients with and without small intestinal bacterial overgrowth and differences in the diversity of species between healthy controls and patients with systemic sclerosis. Trends were also observed in anticentromere antibody systemic sclerosis patients, including higher Alistipies indistincus spp. levels associated with increased methane levels of breath gas testing and higher Slakia spp. levels associated with increased rates of fecal soiling. Conclusions: Our results suggest that changes to the fecal microbiome occur in patients with small intestinal bacterial overgrowth and systemic sclerosis when compared to healthy controls. As a cross-sectional study, the potential pathophysiologic role of an altered microbiome in the development of systemic sclerosis was not considered and hence needs to be further investigated.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2021-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/23971983211032808","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43393955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}