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Population prevalence and symptom associations of autonomic dysfunction in primary biliary cirrhosis
Author(s) -
Newton Julia L.,
Hudson Mark,
Tachtatzis Phaedra,
Sutcliffe Kathryn,
Pairman Jessie,
Burt Jennifer A.,
Jones David E. J.
Publication year - 2007
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.21609
Subject(s) - primary biliary cirrhosis , medicine , population , gastroenterology , environmental health
Abstract Patients with primary biliary cirrhosis (PBC) frequently experience significant fatigue thought to result from as‐yet‐unidentified central nervous system (CNS)‐mediated processes. Pilot studies have suggested that autonomic dysfunction is a frequent occurrence in PBC and may contribute to the pathogenesis of this fatigue. The degree to which autonomic dysfunction affects the PBC population as a whole, and its interrelationship with other symptoms experienced by PBC patients remains unstudied. In this study, we used a geographically defined, fully representative PBC patient cohort to study the prevalence of symptoms of autonomic dysfunction and its relationship with other symptoms of PBC. Symptoms of cardiovascular autonomic dysfunction (as assessed using the Orthostatic Grading Scale [OGS]) were significantly more frequently reported and significantly more severe in PBC patients than in both matched normal controls (40% versus 6% with moderate or worse orthostasis ( P < .0001), mean OGS score 3.2 ± 3.4 versus 1.3 ± 1.9, P < .005) and in patients with primary sclerosing cholangitis and in severity were independently associated with severity of fatigue and cognitive symptoms (both r 2 = 0.3, P < .0001). Thirteen of 20 patients with an OGS value > 4 (moderate severity and worse) had significant abnormality in autonomic regulation of blood pressure, which was identified on dynamic testing. Conclusion: Symptoms suggestive of autonomic dysfunction frequently occur in PBC patients and reflect dysregulation of actual blood pressure. Autonomic dysfunction is independently associated with both fatigue and, importantly, symptoms of cognitive dysfunction, suggesting the potential for significant organic sequelae. (H EPATOLOGY 2007;45:1496–1505.)