Cardiovascular autonomic dysfunction in familial Mediterranean fever.

Abstract

To compare the hemodynamic responses to autonomic challenge evoked by upright tilt table testing in patients with familial Mediterranean fever (FMF). Forty consecutive patients with FMF and 25 age and sex matched healthy controls were evaluated using the head-up tilt test (HUTT). The main outcome measures were the values of blood pressure (BP) and heart rate (HR) recorded during recumbence and tilt. The endpoints of vasodepressor and cardioinhibitory reactions, orthostatic tachycardia, and postural tachycardia syndrome were recorded. Patients with FMF exhibited significantly higher diastolic BP during supine and tilt measurements (p = 0.003 and 0.04, respectively). In response to tilt, patients showed significant increases in HR compared to healthy subjects (p = 0.02). Pathological endpoints on tilt were observed in the FMF group in 7 patients (17%) and in no controls. FMF severity, genotype, duration of illness, response to therapy, and associated amyloidosis did not correlate with pathological reactions on HUTT. FMF patients exhibit an abnormal cardiovascular reactivity, which is clinically occult, but can be detected on autonomic challenge. The abnormal autonomic activity in FMF is similar to dysautonomia described in a variety of rheumatic disorders.