Intensive plasmapheresis as an alternative therapy for intractable pruritus of primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic disease of presumed autoimmune etiology, generally associated with other systemic abnormalities such as scleroderma, characteristic of Sjogren's syndrome and Raynaud's syndrome, for which pruritus is the most troublesome symptom. Treatment of this disease is a major unsolved problem. Although the use of cholestyramine has been effective, a considerable number of cases are refractory to the drug and to other agents such as corticosteroids, azathioprine and penicillamine. Plasma exchange has proven to be a useful option in four female patients with primary biliary cirrhosis--two with grade III histology and the other two with grade IV disease and intractable pruritus. The procedure was well tolerated and no side effects were observed. There was a temporary but significant attenuation of pruritus and improvement of melanoderma. Intensive plasma exchange is proposed as an alternative therapy in primary biliary cirrhosis with refractory pruritus.