Background/Purpose: Studies of severe gastrointestinal (GI) disease in systemic sclerosis (SSc) are limited by small, selected samples composed largely of subjects with prevalent disease. We undertook this study to examine the morbidity and mortality associated with severe GI disease in SSc in a large, unselected inception cohort.  

Methods: A retrospective cohort of subjects with < 3 years of disease duration and without severe GI disease at baseline study visit was identified from the Canadian Scleroderma Research Group registry. Severe GI disease was defined using a previously published definition (Steen and Medsger, A and R 2000) as follows: malabsorption, hyperalimentation, pseudo-obstruction, or either antibiotics for bacterial overgrowth or esophageal stricture requiring dilatation with ≥10% associated weight loss. Subjects who developed severe GI disease during follow-up were compared to those who did not using descriptive statistics. Morbidity was assessed with the Medical Trust Short Form 36 (SF-36) physical (PCS) and mental (MCS) component summary scores. Mortality rates between subjects with and without severe GI disease were compared by dividing the number of deaths per person-years of observation in each group.

Results: Data was available for 306 subjects with < 3 years of disease duration; of these, 21 (7%) had severe GI disease at baseline study visit. Severe GI disease over a mean follow-up time of 3.8 years developed in an additional 18% (50/285) of subjects: 84% female, mean age 53 years, mean disease duration at baseline 1.6 years and proportion with limited/diffuse cutaneous SSc 54%/46%. Subjects who developed severe GI disease were more likely to have digital ulcers (52.0% vs 35.7%, p=0.03) and worse skin scores (15.7 ± 12.0 vs 12.1±10.9, p=0.003) at baseline study visit. Physical health status was more impaired at baseline in subjects with compared to without severe GI disease (SF-36 PCS 35.4 ± 11.3 vs 39.0 ± 10.9, p=0.05). There were no difference in mental health status between the 2 groups at baseline (SF-35 MCS 47.1 ± 11.3 vs 48.6 ± 12.2, p=0.30). There were 10 deaths in 137 person-years of observation among those with, compared to 21 in 1040 person-years of observation among those without severe GI disease, for a mortality rate of 3.7.  

Conclusion: Severe GI disease was common in this inception cohort, affecting approximately 25% of subjects within the first 5 years of disease. It was associated with considerable impairment in physical health status and a striking increase in the risk of mortality. Baseline predictors of severe GI disease included markers of more severe vasculopathy and fibrosis. These findings provide compelling evidence to identify interventions that target selected pathophysiological pathways to reduce the burden of severe GI disease in SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/severe-gastrointestinal-disease-in-early-systemic-sclerosis-is-associated-with-an-increased-risk-of-mortality/