Objective: Light-to-moderate liver damage is often seen in children diagnosed with celiac disease, but severe liver damage is rarely observed.
Methods: During a 12-year-long period our center took care of six 13-36-month-old girls who developed severe liver damage 1-24 months after the diagnosis of celiac disease.
Results: Four girls had acute liver failure; two of them had to be liver transplanted. The other four girls recovered without transplantation and none of the six patients developed autoimmune disease during the 2-14-year-long follow-up period. Although adenovirus type 2 was found in the urine and stools of one girl, her liver histopathology did not resemble viral hepatitis. Certain autoimmune features could be observed initially in some of the children but finally none of them fulfilled the criteria for autoimmune liver disease and this pattern did not change during the several years of follow-up. Thorough investigation could not find any alternative pathogenetic cause and thus, the association with celiac disease is obvious. Histopathology showed various degrees of intralobular inflammation, necrosis, involvement of the small bile ducts, and in one case interface hepatitis; but in general, histopathology did not reveal a common pathogenetic mechanism.
Conclusion: Although rare, severe hepatic damage or failure can develop in association with celiac disease. The etiology is varying and multifactorial. Consequently, children with newly onset celiac disease should be routinely checked for liver function and vice versa, children with severe liver damage should be investigated for untreated celiac disease.