Evaluating the associations between dysautonomia, gastrointestinal transit, and clinical phenotype in patients with systemic sclerosis

Objective Our objective was to identify patients with systemic sclerosis (SSc) with a high burden of autonomic symptoms and to determine whether they have a distinct clinical phenotype, gastrointestinal (GI) transit, or extraintestinal features. Methods In a prospective cohort of patients with SSc with GI disease, clinical data were systematically obtained at routine visits. Dysautonomia was identified by the Composite Autonomic Symptom Score (COMPASS)‐31questionnaire. GI transit was measured by whole‐gut scintigraphy. Associations between total COMPASS‐31 scores and clinical features, GI symptoms, and transit were evaluated. Comparisons between patients with global autonomic dysfunction (GAD; ≥5 positive COMPASS‐31 subdomains) and those with limited autonomic dysfunction (LAD; <5 positive subdomains) were also studied. Results A total of 91 patients with SSc and GI involvement were included (mean age 57 years, 90% female, 74% limited cutaneous disease, 83% significant GI disease Medsger score ≥2). The mean COMPASS‐31 score in patients with SSc was higher than controls (38.8 vs 7.2); 33% had GAD, and 67% had LAD. Patients with GAD were more likely to have limited SSc (93% vs 65%; P < 0.01) and have sicca symptoms (100% vs 77%; P = 0.01). Gastric and colonic transit were faster in patients with GAD ( P < 0.05). Upper GI involvement (Medsger GI score of 1 or 2) was associated with higher total COMPASS‐31 scores ( P = 0.02). Conclusion Symptoms of global dysautonomia are seen in up to one‐third of patients with SSc and GI involvement. Identifying specific clinical characteristics associated with GAD in SSc will help to identify a population that may benefit from therapies that modulate the autonomic nervous system. image