Female patient, 31 years old, smoker of 10 packs/year, treated with oral contraceptives intermittently in the last 8 years up to 1 year before, who presented in October 1997 a 1-month history of nausea and constipation.
Physical examination revealed bilateral palm erythema, jaundice and hepatomegaly.
The imaging tests (ultrasound and abdominal CT) revealed a homogeneous hepatomegaly.
Analyses showed: GOT 384 IU/L, GPT 496 IU/L, GGT 78 IU/L, FA 553 IU/L and bilirubin 11.5 mg/dl (direct with IgG30 mg/dl).
Serology for HAV, HBV and HCV were negative.
ANA were negative.
AMA were positive (1/1280), as well as ASMA (1/320), Anti-LKM1 (1/640) and ANCA (1/40).
A liver biopsy was performed, which showed mild focal distortion due to fibro-portal bands with lymphocytic inflammatory infiltrate in the biliary tracts caused by lymphoid follicles, occasionally.
This infiltrate surpassed the limits of the portal space, necrosis "crying" moderate/severe.
Hepatocytes were observed around the portal space with accumulation of copper-binding protein, and increased inflammatory cellularity in the lobule, both in sinusoids and parenchyma, with fibrotic cytolysis and increased interstitial collagen.
Treatment was initiated with 30 mg prednisone with subsequent dose reduction, 50 mg azathioprine and ursodeoxycholic acid.
There was complete resolution of the clinical picture with normalization of the analysis, persisting slightly elevated, after six years of evolution, AF and GGT.
