[Clinical characteristics of primary biliary cirrhosis: a report of 45 cases].
OBJECTIVE
To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease.


METHODS
General status, clinical manifestations and laboratory findings of 45 patients with PBC were reviewed.


RESULTS
Among the 45 patients, 42 were females and the mean age at diagnosis was (50.8 +/- 8.1) years. The mean time interval between the first visit to physicians to the time of correct diagnosis was about 2 years. The most frequent symptoms were fatigue (66.7%, 30/45), then jaundice (55.6%, 25/45) and pruritus (40%, 18/45). Nine patients (20%) were asymptomatic. Nine patients (20%) were associated with other auto-immune diseases (Sjogren's syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (gamma-GT) levels were markedly elevated in all the patients [(498.5 +/- 369.8) IU/L and (595.2 +/- 518.4) IU/L, respectively], whereas ALT and AST levels were mildly to moderately elevated [(83.9 +/- 58.8) IU/L and (100.8 +/- 48.8) IU/L, respectively]. 25 patients (55.6%) had a total bilirubin level >/= 34.2 micromol/L. 40 patients (88.9%) had elevated serum IgM and 43 patients (95.6%) were anti-mitochondrial antibody (AMA)/AMA-M2 positive.


CONCLUSIONS
In China, PBC is probably not so rare as it was thought before. It is mostly found in middle-aged women. The most frequent symptom is fatigue and some patients are asymptomatic at early stage. Elevated serum ALP and gamma-GT levels together with positive AMA/AMA-M2 can help to diagnose PBC. Liver biopsy is useful to confirm the diagnosis and differentiate histopathological stages.
Zhang
Fukui
F
Liver Research Center, Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.
Jia
Jidong
J
Wang
Baoen
B
Qian
Linxue
L
Yin
Shanshan
S
Wang
Yu
Y
Cui
Yan
Y
You
Hong
H
Ma
Hong
H
Wang
Huiji
H
Zhang
Changhuai
C
D000328:Adult; D000368:Aged; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D000626:Aminopeptidases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D043384:Glutamyl Aminopeptidase; D019694:Hepatitis B, Chronic; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D005723:gamma-Glutamyltransferase / Q000097:blood
D005723:gamma-Glutamyltransferase; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase; D000626:Aminopeptidases; D043384:Glutamyl Aminopeptidase; D001663:Bilirubin