PBC more commonly affects middle-aged (fifth and sixth decades) women than men. Comorbidity with other autoimmune diseases, familial incidences, past or present smoking habits, history of urinary tract infection, and repetitive use of cosmetic products such as hair dyes are significant risk factors for PBC. The clinical features and natural history of PBC significantly vary from asymptomatic to progressive among patients. Jaundice, cholestasis-induced pruritus, and general fatigue are typical symptoms in patients with PBC, although more than half of these patients may have no clinical symptoms (asymptomatic PBC). The prognosis of PBC often depends on the development of portal hypertension or cirrhosis, indicating liver failure. However, disease progression can be significantly inhibited by ursodeoxycholic acid treatment in some patients. Patients with end-stage liver failure require organ transplants. With respect to the racial variation of clinical characteristics, recent nationally representative study in the USA demonstrated the following: (1) Hospitalization rates for PBC was higher among females, Hispanics and Whites; (2) Younger age and low-income earning are predictors of PBC hospitalizations for non-Whites; (3) Mortality was highest in Blacks.
Author(s) Details:
Toru Shizuma,
Department of Physiology, Tokai University School of Medicine, Japan.