Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome?

Objective. We report the cases of two patients with the complete CREST vari ant (calcinosis. Raynaud's phenomenon, oesophageal dysmotility, sclerodacty ly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune he patitis. Results. Our findings suggest that autoimmune hepatitis can be considered t o be one of the liver manifestations associated with SSc. Our data also ind icate that, because liver involvement may precede skin manifestations, eval uation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination. testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capilla roscopy. Conclusions. From a practical point of view, our two cases emphasize that s uspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal manageme nt.