Prevalence, clinical characteristics, and hospital course of systemic sclerosis-associated pseudo-obstruction

Gastrointestinal involvement is almost universal in patients with systemic sclerosis (SSc). Enteric dysmotility, at its most severe, can present with pseudo-obstruction. In this study, we aimed to quantify the prevalence of SSc pseudo-obstruction and evaluate risk factors for its development. In a subgroup of patients requiring admission to hospital for acute episodes of pseudo-obstruction, we evaluated the clinical course and treatments administered.

Using data from 1969 Australian Scleroderma Cohort Study (ASCS) participants, we performed multivariable logistic regression analysis to identify SSc-specific risk factors for pseudo-obstruction. Descriptive statistics were used to examine the clinical course of patients admitted with pseudo-obstruction at a single ASCS centre.

Pseudo-obstruction occurred uncommonly, affecting 70 (3.56%) ASCS participants. Records of 14 participants with a total of 39 admissions for acute pseudo-obstruction were identified. Pseudo-obstruction was associated with longer disease duration (OR 1.03, p = 0.03), bowel dysmotility (OR 4.51, p < 0.01), small intestinal bacterial overgrowth (OR 2.81, 95% CI (1.00-1.05), p < 0.01), and gastric antral vascular ectasia (OR 2.52, 95% CI 1.28-4.94, p < 0.01). Severe diarrhoea, as measured by the UCLA Gastrointestinal 2.0 questionnaire, was the only clinical symptom significantly associated with episodes of pseudo-obstruction (OR 3.39, 95% CI 1.56-7.38, p < 0.01). Opioids were more commonly prescribed in patients with pseudo-obstruction but were not significantly associated with pseudo-obstruction in multivariable analysis (OR 1.24, 95% CI 0.62-2.48, p = 0.54). Patients with a history of pseudo-obstruction were more likely to require enteral (4.29% vs. 0.21%, p < 0.01) or parenteral nutrition (7.14% vs. 0.16%, p < 0.01).

Pseudo-obstruction is associated with other severe gastrointestinal manifestations and is associated with malnutrition in SSc patients. Future studies are required to assess the impact of treatment of SSc-associated enteric dysmotility to prevent progression to pseudo-obstruction. Key Points • Pseudo-obstruction is an uncommon manifestation of systemic sclerosis but frequently recurs and is associated with increased mortality. • Severe diarrhoea and long disease duration are associated with an increased risk of pseudo-obstruction. • Pseudo-obstruction occurs more commonly in patients with severe enteric dysmotility and gastric antral vascular ectasia (GAVE).