Primary biliary cirrhosis is associated with almost every known or postulated autoimmune condition. Collagen diseases, especially Sjogren's syndrome, rheumatoid arthritis, and thyroid disorders are particularly common. Pulmonary abnormalities include lymphocytic interstitial pneumonitis. Gas-transfer studies may be abnormal. Chest radiograms may show nodules and interstitial fibrosis.
Mediastinal lymphadenopathy is common.
The course of PBC is variable and unpredictable. Some patients never become symptomatic, and others run a progressively At present, the patient with end-stage PBC faces possible liver transplantation. A rise in serum bilirubin level is a bad prognostic sign. The Mayo model depends on age, serum bilirubin, and albumin levels, prothrombin time, and edema. Serum bilirubin level and the Mayo model are very good predictors of survival.