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Autonomic Dysfunction Common in Systemic Sclerosis

J Rheumatol; ePub 2018 Jun 15; Adler, Russell, et al

Symptoms of autonomic dysfunction are common in systemic sclerosis (SSc), according to a recent study, and patients with more severe gastrointestinal (GI) disease in SSc report more symptoms of dysautonomia across many facets of the autonomic nervous system. Patients with SSc (n=104) were consecutively recruited during routine clinical visits at the Johns Hopkins Scleroderma Center and asked to complete the Composite Autonomic Symptom Score (COMPASS)-31 questionnaire, a validated tool to assess symptoms of autonomic dysfunction. Researchers found:

  • The mean COMPASS-31 score in this cohort was 24.9 ± 15.5, higher than COMPASS-31 scores from previously published healthy controls (8.9 ± 8.7).
  • Compared to patients with mild or absent GI disease, patients with significant GI disease had higher scores across several subdomains of the COMPASS-31, including orthostatic intolerance (median 10.0 vs 0) and secretomotor dysfunction (median 6.4 vs 4.3).
  • There was also a dose-response relationship between GI disease severity and autonomic symptom burden.


Adler BL, Russell JW, Hummers LK, McMahan ZH. Symptoms of autonomic dysfunction in systemic sclerosis assessed by the COMPASS-31 Questionnaire. [Published online ahead of print June 15, 2018]. J Rheumatol. doi:10.3899/jrheum.170868.