Devic's neuromyelitis optica and autoimmune hepatitis: first description of a new association.

Abstract We the report the case of a 28 years old female patients which referred to our Department in May 2005 for acute hepatitis and a history of relapsing neurologic deficits. Transverse myelitis was diagnosed and she was treated with corticosteroids obtaining progressive remission. Two months thereafter she complained a right sided visual loss. Optic neuritis was diagnosed and she was treated with corticosteroids again, obtaining a significant improvement. A diagnosis was made of autoimmune hepatitis (AIH) in a patient affected by neuromyelitis optica (NMO). She was treated with corticosteroids, IVIg and azathioprine, with progressive remission. No new flares of hepatic or neurologic disease were observed in the 2 years follow-up , except for residual visual deficit. NMO (also known as Devic’s syndrome) is a demyelinating disease whose distinction from multiple sclerosis (MS) has long been unclear, because NMO patients usually fulfill MS diagnostic criteria. The importance of separating NMO from MS is twofold: 1) NMO has a worse outcome but 2) responds to immunosuppressive therapy. NMO has been reported in association with a widespread group of immune-mediated diseases, including SLE, Sjogren’s syndrome, vasculitis and sarcoidosis. To date, this is the first description on NMO and AIH occurring in the same patient.