Summary
This study explores the prevalence and risk factors for “autonomic dysfunction” in scleroderma. The autonomic nervous system controls bodily functions such as breathing, blood pressure, the heartbeat, and digestive processes. We find that symptoms of autonomic dysfunction are common in scleroderma, and that gastrointestinal (GI) dysfunction tends to occur with other symptoms of autonomic dysfunction including dizziness and sweating disturbances.
Why was the study done?
Patients with scleroderma are known to have autonomic dysfunction including impaired heart rate and blood pressure control, GI disturbances, and sweating dysfunction. Prior studies in this area are small and have been unable to identify risk factors that are associated with autonomic dysfunction in scleroderma. The goal of this study was to measure the severity of autonomic symptoms among scleroderma patients and understand if specific clinical or lab features are associated with autonomic dysfunction.
How was the study done?
We administered a questionnaire called the COMPASS-31 to 104 scleroderma patients seen at the Johns Hopkins Scleroderma Center. The COMPASS-31 is a tool that is used to measure autonomic symptoms across multiple aspects of the autonomic nervous system. This includes questions about dizziness, sweating dysregulation, Raynaud’s phenomenon, GI dysfunction, pupil dysfunction, and urinary dysfunction. We then determined whether there were any clinical or laboratory features that were associated with symptoms of autonomic dysfunction in patients with scleroderma.
What were the major findings?
We found that symptoms of autonomic dysfunction are common among patients with scleroderma. This included patients with both limited and diffuse skin disease, and there was no particular autoantibody that predicted autonomic dysfunction. The symptoms also spanned across multiple facets of the autonomic nervous system. The burden of autonomic symptoms observed in patients with scleroderma was comparable to other diseases that primarily affect the autonomic nervous system such as diabetes. We found that GI dysfunction in scleroderma was strongly associated with symptoms of dizziness as well as dysfunction of the sweat glands and temperature regulation.
What is the impact of this work?
This study shows that scleroderma patients have a significant burden of symptoms related to autonomic dysfunction. There were no particular clinically or lab features that predicted who would develop these symptoms, which suggests that all patients with scleroderma should be screened for this complication in routine clinical practice. The COMPASS-31 tool may be a useful questionnaire for assessing the burden of autonomic symptoms in patients with scleroderma. GI dysfunction in scleroderma was strongly associated with other symptoms of autonomic dysfunction, suggesting that future studies are needed to understand the role of the autonomic nervous system in the pathogenesis of scleroderma.
This research was supported by:
Research supported by the Rheumatology Research Foundation Scientist Development Award, the Jerome L. Greene Foundation (90057213), the Scleroderma Research Foundation (ZHM), the Johns Hopkins Clinician Scientist Research Career Development Award (ZHM), and the US National Institute of Arthritis and Musculoskeletal and Skin Diseases (BLA, T32AR048522). JWR was supported in part by the US National Institute of Diabetes and Digestive and Kidney Diseases, the National Institutes of Health and Office of Research Development (1R01DK107007-01A1), the Department of Veterans Affairs (Biomedical and Laboratory Research Service and Rehabilitation Research and Development, 101RX001030).
Link to the original research publication:
Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed by the COMPASS-31 Questionnaire. Adler BL, Russell JW, Hummers LK, McMahan ZH. J Rheumatol. 2018 Jun 15. pii: jrheum.170868. doi: 10.3899/jrheum.170868.
