Autoimmune chronic active hepatitis and primary biliary cirrhosis.

Autoimmune CAH is important to recognize, since it is highly responsive to treatment which undoubtedly prolongs life. Autoimmune CAH can rarely be cured; complete withdrawal of treatment leads to relapse in over 80% of patients. Prednisolone and azathioprine are the major drugs of choice, the former inducing remission while the latter maintains remission, either alone or in combination with prednisolone. Since both drugs are associated with substantial side-effects which tend to be dose-related, the object of treatment must be to induce and maintain remission with the minimum risk of relapse together with an acceptably low incidence of complications. Although PBC shares many features in common with autoimmune CAH, treatment of the underlying disease is generally unsuccessful. To date no drug has been shown to induce remission or to prolong survival. The main aim of treatment should be directed towards the complications of PBC, of which pruritus and osteoporosis are the two major complaints. Cholestyramine and antihistamines are the drugs of choice, but when these fail a variety of other therapies are also available, although many have only been shown to be effective on an anecdotal basis. No treatment has yet been shown to reverse the bone demineralization which occurs in PBC, but early calcium supplementation is recommended in this disorder. Osteomalacia is uncommon and can be prevented by prophylactic calcium and vitamin D supplementation in jaundiced patients. Liver transplantation is effective in treating PBC, and when successful leads to complete restoration of health with the prospects of increasingly long survival. Recurrence of PBC does not appear to be a significant problem.