We present the case of dual adult celiac disease and liver disease with por
tal hypertension (esophageal varices); a percutaneous liver biopsy was comp
atible with nonspecific reactive hepatitis. Clinically, celiac disease was
characterised by poor response to a gluten-free diet, with the development
of a biochemical cholestasis and marked malnutrition. Our patient died of c
erebral hemorrhage, at the age of 50 years, without associated risk factors
. The necropsy demonstrated the existence of a nodular regenerative hyperpl
asia of the liver, splenic atrophy, gelatinous transformation of the bone m
arrow, and lymphocytic colitis. We discuss the different types of liver dis
orders associated with celiac disease and the possible relation between nod
ular regenerative hyperplasia and celiac disease, based on immunologic mech
anisms.