Severe gastrointestinal disease linked to increased mortality in early scleroderma
Severe gastrointestinal (GI) involvement is common in early systemic sclerosis and is associated with an elevated risk of mortality and significant impairment in health-related quality of life, a study reports.
The study included 556 systemic sclerosis patients (311 Canadian and 245 Australian; mean age 54.0 years; 80.8 percent female) with <2 years of disease duration. Severe GI disease was defined as malabsorption, hyperalimentation, pseudo-obstruction and/or ≥10 percent weight loss in relation to the use of antibiotics for bacterial overgrowth or oesophageal stricture.
Researchers performed Kaplan–Meier, multivariate logistic regression and Cox proportional hazard analyses to determine the cumulative incidence rate, independent clinical correlates and mortality rate associated with severe GI disease.
In the population, 26 patients (4.7 percent) had severe GI disease at baseline while 46 (8.3 percent) developed it over a mean follow-up of 4.3 years. The probability of developing the disease was 9.1 percent at 2 years and 16.0 percent at 4 years. Risk factors included inflammatory myositis (odds ratio [OR], 4.68; 95 percent CI, 1.65–13.24), telangiectasias (OR, 2.45; 1.19–5.04) and modified Rodnan skin score (OR, 1.03; 1.01–1.07).
Severe GI disease was associated with a greater than twofold increase in the risk of death (hazard ratio, 2.27; 1.27–4.09) and worse health-related quality of life (Short Form Health Survey physical and mental component summary scores, p=0.02 and p=0.01, respectively).
The findings indicate that myositis is the strongest independent predictor of severe GI disease and suggest that a common pathway might be targeted to address both the skeletal muscle and intestinal smooth muscle involvement, researchers said. More studies should be conducted to further the understanding of this serious manifestation of systemic sclerosis, as well as to identify preventive strategies.