ABSTRACT
Primary biliary cirrhosis (PBC) is likely an autoimmune disease that destroys the
interlobular bile ducts. Although the term PBC implies cirrhosis, this is not always
present. The condition may be entirely silent clinically, save for the hallmark mitochondrial
antibodies in serum. The clinical spectrum of PBC ranges from asymptomatic anicteric
cholestasis with or without extrahepatic manifestations to severe cholestasis with
decompensated cirrhosis. It is uncertain whether or not the course of this disease
is universally fatal. Currently, no specific features have been identified which predict
progression from asymptomatic to symptomatic disease, although once hyper-bilirubinemia
is present, a rising level indicates a poor prognosis. The liver-specific complications
include pruritus, abdominal pain, xantholasma, and portal hypertension. The latter
is often an early feature, as the portal hypertension is presinusoidal in nature and,
when present, does not always reflect the presence of cirrhosis. There are many extrahepatic
features of PBC, the most common being metabolic, chiefly hypothyroidism and metabolic
bone disease. Other common associations are rheumatologic, renal, pulmonary, neuromuscular,
and dermatologic. The nonspecific yet distressing symptom of fatigue affects up to
two-thirds of PBC subjects, but its etiology remains obscure.
KEY WORDS
asymptomatic - symptomatic - clinical features - natural history
