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J Rheumatol. 2018 Aug;45(8):1145-1152. doi: 10.3899/jrheum.170868. Epub 2018 Jun 15.

Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire.

Author information

1
From the Department of Rheumatology, Johns Hopkins University School of Medicine; the departments of Neurology, Anatomy, and Neurobiology, University of Maryland School of Medicine; the Veterans Affairs Medical Center, Baltimore, Maryland, USA. badler6@jhu.edu.
2
B.L. Adler, MD, Rheumatology Fellow, Department of Rheumatology, Johns Hopkins University School of Medicine; J.W. Russell, MD, Professor of Neurology, departments of Neurology, Anatomy and Neurobiology, University of Maryland School of Medicine and Veterans Affairs Medical Center; L.K. Hummers, MD, MSc, Associate Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine; Z.H. McMahan, MD, MHS, Assistant Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine. badler6@jhu.edu.
3
From the Department of Rheumatology, Johns Hopkins University School of Medicine; the departments of Neurology, Anatomy, and Neurobiology, University of Maryland School of Medicine; the Veterans Affairs Medical Center, Baltimore, Maryland, USA.
4
B.L. Adler, MD, Rheumatology Fellow, Department of Rheumatology, Johns Hopkins University School of Medicine; J.W. Russell, MD, Professor of Neurology, departments of Neurology, Anatomy and Neurobiology, University of Maryland School of Medicine and Veterans Affairs Medical Center; L.K. Hummers, MD, MSc, Associate Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine; Z.H. McMahan, MD, MHS, Assistant Professor of Medicine, Department of Rheumatology, Johns Hopkins University School of Medicine.

Abstract

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.

KEYWORDS:

AUTONOMIC DYSFUNCTION; GASTROINTESTINAL TRACT; NEUROLOGIC MANIFESTATIONS; SELF-ASSESSMENT; SYSTEMIC SCLEROSIS

PMID:
29907667
PMCID:
PMC6072589
[Available on 2019-08-01]
DOI:
10.3899/jrheum.170868

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