AB1033 Evidence for Autonomic Dysfunction in Familial Mediterranean Fever (FMF): Results from a Prospective Controlled Study Including the Compass-31 Questionnaire

BackgroundFamilial mediterranean fever (FMF) is the most prevalent hereditary autoinflammatory disorder. While disease features such as fever, polyserositis and systemic amyloidosis are well recognized, little is known about the involvement of the autonomous nervous system. However, autonomic dysfunction has been identified as a major problem in autoimmune diseases such as systemic lupus erythematosus and diabetes mellitus (1). Resulting morbidities include orthostatic and gastrointestinal dysregulation and reduced heart rate variability.ObjectivesTo prospectively investigate whether signs and symptoms of autonomic dysfunction are more prevalent in FMF patients than in age and gender matched healthy controls.MethodsPatients fulfilling the classification criteria for FMF were included in the study. Data on genotype, disease activity, disease manifestations, current treatment and presence/absence of amyloidosis was gathered. Patients and healthy controls were evaluated using the COMPASS-31 questionnaire, a well-validated and feasible self-assessment instrument for the evaluation of autonomic functions and symptoms (2). Additional tests included assessment of blood pressure responses to sympathic and parasympathic stimuli, heart rate variability analysis and skin conductance measurement.ResultsSofar, 18 FMF patients and 15 healthy controls were enrolled into this ongoing study. Mean age and gender distribution did not differ significantly in both groups. Compared to healthy controls, FMF patients had a significantly higher mean COMPASS-31 score (20.7±6.8 vs. 7.1±5.3, p