PT  - JOURNAL ARTICLE
AU  - Brittany L.  Adler
AU  - James W.  Russell
AU  - Laura K.  Hummers
AU  - Zsuzsanna H.  McMahan
TI  - Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire
AID  - 10.3899/jrheum.170868
DP  - 2018 Jun 15
TA  - The Journal of Rheumatology
PG  - jrheum.170868
4099  - http://www.jrheum.org/content/early/2018/06/11/jrheum.170868.short
4100  - http://www.jrheum.org/content/early/2018/06/11/jrheum.170868.full
AB  - Objective Autonomic dysfunction is a known complication of systemic sclerosis (SSc) that can affect vascular tone, gastrointestinal (GI) motility, heart rate, and blood pressure control. We sought to quantify autonomic symptom burden in SSc, and to define the characteristics of patients with SSc and autonomic dysfunction. Methods Patients with SSc 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. We determined the relationship between various clinical and serological features of SSc and the total COMPASS-31 scores and domain-specific scores using the Student t test or Wilcoxon rank-sum test for dichotomous variables and linear regression analysis for continuous variables. Results The study included 104 patients with SSc who completed the COMPASS-31 questionnaire. 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, p = 0.006) and secretomotor dysfunction (median 6.4 vs 4.3, p = 0.03). There was also a dose-response relationship between GI disease severity and autonomic symptom burden. Conclusion Symptoms of autonomic dysfunction are common in SSc. Patients with more severe GI disease in SSc report more symptoms of dysautonomia across many facets of the autonomic nervous system.