Background/Purpose:

Autonomic dysfunction is a known complication of systemic sclerosis (SSc) and can affect vascular tone, gastrointestinal (GI) motility, and heart rate and blood pressure control. We sought to quantify autonomic symptom burden in a large cohort of patients with SSc and to determine clinical and serologic risk factors that associate with autonomic symptom burden.

Methods:

Patients with SSc were recruited from the Johns Hopkins Scleroderma Center and completed the Composite Autonomic Symptom Score (COMPASS)-31 questionnaire, which is a validated tool to measure autonomic symptom burden. The COMPASS-31 scores and subdomain scores in orthostatic, secretomotor, vasomotor, gastrointestinal, urinary, and puillomotor dysfunction were quantified for each patient. Demographic information, clinical and serologic data, and Medsger disease severity scores were obtained from the longitudinal Johns Hopkins Scleroderma Center database. We analyzed the relationship between various features of systemic sclerosis and the total COMPASS-31 scores and subdomain scores using the student’s t-test for dichotomous variables and linear regression analysis for continuous variables.

Results:

104 patients with SSc completed the COMPASS-31 questionnaire. The average COMPASS-31 score among our SSc cohort was 24.9 ± 15.5, which was comparable to previously published scores in other diseases known to affect the autonomic nervous system such as small-fiber polyneuropathy. There was no relationship between the COMPASS-31 score and SSc subtype or autoantibody status. Patients with severe GI disease reported higher scores in multiple subdomains of the COMPASS-31, including orthostatic intolerance (p=0.006) and secretomotor dysfunction (p=0.03), compared to patients with mild or absent GI disease. There was also a dose-response relationship between GI disease severity and autonomic symptom burden (see Figure).

Conclusion:

We determined that patients with SSc have a significant burden of autonomic symptoms across multiple domains of autonomic function. All patients with SSc are at risk for autonomic symptoms regardless of SSc subtype or autoantibody status. We also determined that patients with severe GI disease have more symptoms of dysautonomia, which suggests that some of the GI disease in systemic sclerosis may be a cause or consequence of autonomic dysfunction.