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. 2019 Apr 1;58(4):636-644.
doi: 10.1093/rheumatology/key350.

Severe gastrointestinal disease in very early systemic sclerosis is associated with early mortality

Nicolas Richard  1   2 , Marie Hudson  2   3   4 , Mianbo Wang  4 , Geneviève Gyger  2   3 , Susanna Proudman  5   6 , Wendy Stevens  7 , Mandana Nikpour  7   8 ; Canadian Scleroderma Research Group (CSRG); Australian Scleroderma Interest Group (ASIG); Murray Baron  2   3   4
Affiliations

Severe gastrointestinal disease in very early systemic sclerosis is associated with early mortality

Nicolas Richard et al. Rheumatology (Oxford). .

Abstract

Objectives: To examine the incidence, predictors and outcomes associated with severe gastrointestinal (GI) disease in a large inception SSc cohort.

Methods: SSc subjects with <2 years of disease duration were identified from two multicentre cohorts. Severe GI disease was defined as: malabsorption, hyperalimentation, pseudo-obstruction and/or ⩾10% weight loss in association with the use of antibiotics for bacterial overgrowth or oesophageal stricture. Kaplan-Meier, multivariate logistic regression and Cox proportional hazard analyses were performed to determine the cumulative incidence rate, independent clinical correlates and mortality rate associated with severe GI disease. A longitudinal mixed model was used to assess the impact of severe GI disease on the Short Form Health Survey.

Results: In this inception SSc cohort, the probability of developing severe GI disease was estimated at 9.1% at 2 years and 16.0% at 4 years. In multivariate analysis, severe GI disease was associated with inflammatory myositis (odds ratio 4.68, 95% CI 1.65, 13.24), telangiectasias (odds ratio 2.45, 95% CI 1.19, 5.04) and modified Rodnan skin score (odds ratio 1.03, 95% CI 1.01, 1.07). Severe GI disease was associated with a >2-fold increase in the risk of death (hazard ratio 2.27, 95% CI 1.27, 4.09) and worse health-related quality of life [Short Form Health Survey physical (β = -2.37, P = 0.02) and mental (β = -2.86, P = 0.01) component summary scores].

Conclusion: Severe GI disease is common in early SSc and is associated with significant morbidity and increased mortality. More research is needed to understand, prevent and mitigate severe GI disease in SSc.

Keywords: gastrointestinal manifestations; health-related quality of life; mortality; scleroderma; systemic sclerosis.

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Figures

<sc>Fig</sc>. 1
Fig. 1
Cumulative incidence curve to show time to onset of severe GI disease since the onset of disease (N=556)
See this image and copyright information in PMC

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