RESUMO
Autoimmune hepatitis concomitant with other immune-mediated diseases is an increasingly recognized condition which is difficult to diagnose. We present the case of a 42-year-old woman with no significant medical history who consulted due to progressive growth of an abdominal mass in the right hypochondriac region and associated constitutional syndrome. The physical exam showed hepatomegaly, calcinosis and salt-and-pepper depigmentation of the skin, and Raynaud's phenomenon in the hands. Paraclinical tests reported elevated transaminases and IgM immunoglobulin, as well as positive antinuclear antibodies (ANAs) and smooth muscle antibodies (SMAs), along with imaging signs of portal hypertension. A liver biopsy was compatible with autoimmune hepatitis, and treatment was begun with corticosteroids, with an adequate response. Systemic sclerosis is one of the autoimmune diseases which can present in a patient with autoimmune hepatitis. Suspecting, diagnosing and following up these diseases in this type of patients is key in their comprehensive management. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2609).