Asymptomatic Primary Biliary Cirrhosis

Al, Viteri; Jf, Greene

doi:10.1097/00007611-198002000-00013

Cited by 4 publications

(1 citation statement)

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“…Reports have suggested that certain clinical features may predict the later development of symptomatic disease; those features most commonly cited are the presence of hepatomegaly, the presence of other autoimmune disorders, and the presence of cirrhosis on liver biopsy. [13][14][15][16][17] However, a recent study done by Springer et a1 does not confirm that any clinical features are predictive of subsequent development of symptomatic disease. 18 Although the presence of cirrhosis on biopsy may be found even in those who are asymptomatic, follow-up of patients found to be cirrhotic does suggest that their overall survival is less than those found not to be cirrhotic at first presentation.19 These samc authors reported that the overall survival of asymptomatic patients was the same as an aged-matched population, but later studies indicate that this may not be ~0.18320 This discrepancy is likely due to the fact that the general health of the population has improved in the time between the first study and later studies.…”

Section: The Natural History Of Asymptomatic Pbcmentioning

confidence: 99%

The Clinical Expression of Primary Biliary Cirrhosis

Heathcote¹

1997

Semin Liver Dis

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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 hyperbilirubinemia 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 non-specific yet distressing symptom of fatigue affects up to two-thirds of PBC subjects, but its etiology remains obscure.

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