The development of connective tissue diseases in patients with autoimmune hepatitis: a case series.

Objectives: To study the prevalence of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH).

Methods: We identified 11 cases of AIH over the past 7 years at our institution, through a systematic chart review of patients with this diagnosis. Their charts were reviewed for the development of systemic CTD.

Results: Three of the 11 patients with a definitive diagnosis of AIH developed systemic CTD. All were white: 2 women and 1 man, with an age range of 33 to 62 years, and with disease duration of 1 to 7 years. One patient developed systemic lupus erythematosus (SLE) with vasculitis and peripheral neuropathy. The second developed limited scleroderma and the third developed undifferentiated connective tissue disease (UCTD) and interstitial lung disease. There appear to be shared susceptibility alleles for AIH and CTD in addition to the shared positive autoantibodies.

Conclusions: Patients with AIH may be at increased risk for developing systemic CTD. Conversely, a review of the literature reveals that patients with systemic CTD may be at increased risk of developing AIH. Patients with either AIH or CTD should be monitored for further development of concurrent autoimmune diseases.