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

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.

Spontaneous bowel perforation in the setting of colonic involvement with scleroderma

“…Fibrosis, neurogenic and myogenic dysfunction are mediated by dysregulated immune responses and vasculopathy that occurs in SSc 1. In addition, Richard et al found a strong association between severe GI involvement and inflammatory myositis suggesting a possible common underlying mechanism 3. Interestingly, our patient was diagnosed with myositis for which she required long-term steroid therapy.…”

“…It can manifest as diverticulosis, telangiectasia, constipation, bacterial overgrowth and malabsorptive diarrhoea, chronic intestinal pseudo-obstruction or benign pneumatosis intestinalis 1. Severe GI involvement, associated with increased morbidity and mortality, is reported to occur in about 8%–16% of SSc patients2 3 and was shown to be associated with inflammatory myositis 3. Here we present a case of fatal sigmoid colon perforation in a patient with SSc and myositis.…”

Significant weight loss in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

“…The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease 2. However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc.…”

Comparing Clinical, Imaging, and Physiological Correlates of Intestinal Pseudo-Obstruction: Systemic Sclerosis vs Amyloidosis and Paraneoplastic Syndrome

“…No clear effect of sex on GI involvement in SSc was seen in a previous study, except for constipation, which was more common in women ( 20 ). Another study also did not find SSc-related GI complications to associate with sex ( 21 ). Finally, 1 study showed male sex to be associated with severity of GI dysfunction ( 22 ).…”