Population prevalence & symptom associations of autonomic dysfunction in PBC

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Hepatology

Published Online: 30

May 2007  Copyright © 2007 American Association for the

Study of Liver Diseases

Population prevalence and symptom associations of

autonomic dysfunction in primary biliary cirrhosis

L. Newton, Mark Hudson , Phaedra

Tachtatzis1,

Sutcliffe, Jessie Pairman, A. Burt,

E. J.

Liver Research Group, Newcastle-upon-Tyne,

UK

Cardiovascular Investigation Unit, Newcastle-upon-Tyne,

UK

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 =

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.

(HEPATOLOGY 2007;45:1496-1505.)

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