Primary sclerosing cholangitis with autoimmune hemolytic anemia and pulmonary infiltrations

A 39-year-old man presenting with pulmonary infiltrations and hemolytic anemia was diagnosed as having primary sclerosing cholangitis (PSC) without evidence of ulcerative colitis. This constellation of associations is unique to the best of our knowledge. Autoimmune hemolytic anemia has been reported to be associated with PSC on only a few occasions, and pulmonary infiltrations in association with PSC are also quite unusual. Genetic and immunologic mechanisms are major factors in the pathogenesis of these disorders.