In general, the disease is progressive. Recurrent bacterial cholangitis is treated with antibiotics as needed. Dominant strictures may be managed by endoscopic or transhepatic dilation, with or without stent placement. Corticosteroids, azathioprine, penicillamine, and methotrexate have variable results and are associated with significant toxicity. Ursodeoxycholic acid may reduce itching and improve biochemical parameters but has not been shown to alter the natural history of the disease.
Liver transplantation is the only apparent cure.
The optimal timing of transplantation to prevent this complication is unknown. All rights reserved.