Co-occurrence of idiopathic granulomatous hepatitis and primary biliary cirrhosis

PBC is an autoimmune disease affecting the bile ducts. Granulomas can be found in portal triads in 45 % of patients with PBC. Idiopathic granulomatous hepatitis is a rare disease of unknown cause which is characterized by recurrent fevers, sweats, elevated levels of liver enzyme tests, particularly the serum alkaline phosphatase, and granulomas in the portal and lobular regions of the liver. Previous literature suggests that a diagnosis of idiopathic granulomatous hepatitis can be made only if PBC has been excluded.

We reviewed instances in which PBC and idiopathic granulomatous hepatitis occurred in the same patient.

We report three patients in whom both diseases occurred: 1) A patient with PBC who was diagnosed 15 years later with idiopathic granulomatous hepatitis; 2) A patient with idiopathic granulomatous hepatitis who developed PBC 12 years later; and 3) A patient who had features of both idiopathic granulomatous hepatitis and PBC at the time of initial diagnosis.

Our experience with these patients suggests that idiopathic granulomatous hepatitis and PBC can occur in the same individual. Knowing this association is important, as clinical deterioration in a patient with either disease could suggest the presence of the other and should be treated accordingly.