Summary: Over a period of 16 years There were 7 deaths, three unrelated to liver disease. Survival analysis gives an 86% 5-year survival and 56% 10-year survival. Twenty four patients were treated with steroids (and 6 additionally with azathioprine); 15 (63%) were steroid responsive and 9 were non-responsive. In five patients steroids were successfully discontinued but in 10 patients severe symptomatic relapse occurred on steroid reduction below 7.5-10 mg/day. Steroid non-responders were not typical CAH, 5 with predominantly a rise in alkaline phosphatase, one multisystem disease and one HBV positive. Steroid discontinuation was only possible in one third of the patients responding. Eighteen subjects (69%) were cirrhotic at initial biopsy; 9 had follow-up biopsies, 6 were unchanged but three had progressed to cirrhosis despite apparent steroid responsiveness.