[Clinical analysis of primary biliary cirrhosis: a report of 42 cases]

To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease.

Clinical data of 42 patients clinically and/or histologically diagnosed with PBC were reviewed. Anti mitochondrial antibody (AMA) negative/positive patients as well as the patients who were/were not associated with Sjogren Syndrome (SS) were compared in terms of clinical, biochemical and immunological features.

Among the 42 patients, 78.6% (33/42) of the cases were females; the mean age at diagnosis was (61.1+/-10.8) years. The most frequent symptoms were fatigue. Serum alkaline phosphatase (ALP), gamma-glutamyltranspeptidase (gamma-GT) and total bile acid (TBA) levels were markedly elevated in the majority of the patients, whereas ALT and AST levels were mildly to moderately elevated. Thirty-one patients had a total bilirubin (TBil) level above normal. The levels of TBil and prothrombin time had positive correlationship with years of the course (P=0.000, r=0.696; P=0.005, r=0.424), whereas serum albumin level had negative correlationship with years of the course (P=0.002, r=-0.462). Thirty-seven patients had elevated serum IgM and 34 patients were AMA/AMA-M(2) positive. AMA negative and AMA positive patients were similar in terms of clinical manifestations and liver biochemistries findings. Serum IgM and IgA levels were significantly lower, whereas total cholesterol level was higher in AMA negative patients when compared with AMA positive cases. Fifteen cases were associated with SS, which were similar in terms of clinical, biochemical and immunological features when compared with the PBC patients were not associated with SS.

PBC is mostly found in middle aged and old women. Elevated serum ALP, TBA and gamma-GT levels together with positive AMA/AMA-M(2) can help to diagnose PBC. AMA negative PBC patients are characterized by relatively lower serum IgM and IgA levels and higher total cholesterol level. PBC patients who are associated with SS have not substantial differences in the clinical, biochemical and immunological spectra of the disease.