Fulminant type 1 autoimmune hepatitis in a recently diagnosed celiac disease patient

Celiac disease (CD) is a small intestine immune-mediated disorder triggered by gluten ingestion in genetically predisposed patients. This condition can also affect many extraintestinal tissues, including the liver. We report a patient presenting with a marked increase of transaminases at diagnosis of CD. The immune markers for autoimmune hepatitis (AIH) were negative. Following a few months of a strict gluten-free diet (GFD), aminotransferase levels decreased significantly (< 2.5x U/L). The response to GFD suggested that the liver damage was due to a gluten-dependent celiac hepatitis, the most common liver abnormality in CD. Despite the fact that the patient never stopped the GFD, yet, in a few months, the aminotransferase levels raise again to high values (> 50x U/L). At this time, the liver autoantibodies turned to be positive thus confirming the development of a type 1 AIH. The hepatic damage progressed to a late onset liver failure requiring liver transplantation.