Primary biliary cirrhosis in India.
BACKGROUND
Primary biliary cirrhosis (PBC) is a rare cause of chronic liver disease in India. We analyzed the clinical, biochemical, serological and histological features of patients with PBC for over a 10-year period.


METHODS
PBC was diagnosed by the presence of raised level of serum alkaline phosphatase (ALP), anti-mitochondrial antibody (AMA) positivity (1:40 dilution), and/or diagnostic liver histology.


RESULTS
Fifteen female patients with mean age of 46.5+/-11 years were studied. Pruritus (80%) followed by jaundice (67%), skin changes (pigmentation, coarsening, xanthelesma and vitiligo) (67%) and fatigue (60%) were common symptoms. The mean duration of the symptoms was 3.5+/-5.4 years (3 months to 20 years). Dryness of eyes was observed in only 2 patients. Hepatomegaly was noted in 87% of the patients and ascites at presentation in 40%. Mean levels of bilirubin and albumin at the time of diagnosis were 3.4+/-3.3 mg/dl and 3.5+/-0.8 g/dl, respectively. The level of serum ALP ranged from 54 to 2400 IU/L, with a median being 552 IU/L (2 x ULN). In all the 15 patients with AMA positive, 8 (53%) were also positive for either anti-nuclear or anti-smooth muscle antibodies. Two patients presented with persistently elevated SAP after an acute hepatitic illness. Liver biopsy was available in 13 patients, diagnostic of PBC II & III (8) and with evidence of cirrhosis (5). Associated autoimmune disorders were observed in 5 patients (33%). The mean time for follow-up was 26+/-21 months (1 to 87 months). In 4 deaths, 3 were due to liver related causes.


CONCLUSIONS
PBC is a rare cause of chronic liver disease in India. PBC in India, unlike in the West, presents late, often with features of cirrhosis and decompensation.
Department of Gastroenterology, G.B. Pant Hospital, New Delhi-110002, India. sksarin@nda.vanl.net.in
Shiv Kumar
SK
Sarin
Rajnish
R
Monga
Bimaljeet Singh
BS
Sandhu
Barjesh Chander
BC
Sharma
Puja
P
Sakhuja
Veena
V
Malhotra
D000328:Adult; D000469:Alkaline Phosphatase / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000453:epidemiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D012189:Retrospective Studies
D001323:Autoantibodies; D015415:Biomarkers; D000469:Alkaline Phosphatase