Type 1 autoimmune hepatitis in a patient with microscopic polyangiitis: challenges in diagnosis and treatment

Autoimmune diseases/autoantibodies tend sometimes to cumulate in the same individual, probably as a consequence of defects in immune regulation with breakdown of self-tolerance. Autoimmune hepatitis and microscopic polyangiitis have been occasionally reported with other autoimmune diseases, but the particular association of these both disorders has not been previously reported in the English and Spanish medical literature.

A 72 year-old woman presented with symptoms suggesting giant cell arteritis and polymyalgia rheumatica.

A temporal artery biopsy disclosed a spared temporal artery, with vasculitis involving surrounding small vessels. Anti-neutrophil cytoplasmic antibodies were positive, with myeloperoxidase specificity. Increased liver enzymes led to a wider autoantibody determination. Anti-nuclear antibodies and anti-smooth muscle cell antibodies with anti-f-actin specificity were also positive. A liver biopsy showed changes consistent with autoimmune hepatitis.

Clues for the diagnosis of vasculitis and AIH in the context of this patient, challenges in its classification among systemic vasculitides, and difficulties in the choice of a suitable therapeutic management for this particular association are discussed.