Symptoms of autonomic dysfunction in systemic sclerosis assessed with the COMPASS-31 questionnaire: associations with disease severity and chronic pain

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Introduction Autonomic dysfunction in systemic sclerosis (SSc) mainly influences the cardiovascular system and gastrointestinal motility. Observations to date indicate that it is not a rare complication of SSc. The Composite Autonomic Symptom Score (COMPASS-31) questionnaire assesses the impact of autonomic dysfunction on the quality of life of patients and the dependence on the involvement of specific systems. This study aimed to assess the prevalence and severity of symptoms of autonomic dysfunction in patients with SSc, as well as the relationship between these symptoms and other clinical manifestations of the disease. Material and methods Fifty-five consecutive patients with SSc were included in this study. All patients completed the COMPASS-31 questionnaire, which consists of 31 items, quantifying 6 autonomic domains: orthostatic intolerance (OI), vasomotor dysfunction (VD), secretomotor dysfunction (SD), gastrointestinal dysfunction (GD), urinary bladder dysfunction (BD) and pupillomotor dysfunction (PD). Differences in the total COMPASS-31 score and domain-specific scores were assessed with respect to disease subset, SSc-specific antibodies, capillaroscopic findings, lung diffusing capacity and chronic pain. Furthermore, we assessed the correlation between the COMPASS-31 score and disease status (assessed using the Scleroderma Assessment Questionnaire), and the severity of gastrointestinal (GI) symptoms (assessed using the the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 questionnaire). Results Symptoms of OI were found in 32/55 (58.2%), VD in 49/55 (89.1%), SD in 36/55 (65.5%), GD in 40/55 (72.7%), BD in 26/55 (47.3%) and PD in 30/55 (54.5%) of our patients with SSc. The total COMPASS-31 score did not correlate with age or disease duration. Prevalence of symptoms of diffe­rent autonomic dysfunctions was similar regardless of disease subtype, antibodies or capillarosco­pic findings. Patients with decreased lung diffusing capacity had more frequent and more severe symptoms of GD and BD, and a higher PD score. Patients with chronic pain had more frequent symptoms of OI, SD, GD, BD and PD, than patients without pain. However, prevalence of different autonomic dysfunctions was similar in patients with nociceptive, neuropathic, or mixed pain. We found that patients with more severe disease, including GI involvement, had more common symptoms of autonomic dysfunction. Conclusions Patients with more severe disease, as well as patients suffering from chronic pain, reported more severe symptoms of autonomic dysfunction.