Idiopathic familial cirrhosis and steatosis in adults.

A family was studied in which three middle-aged siblings had unexplained cirrhosis and steatosis. Five of nine additional family members had abnormalities of liver function. Liver biopsy in those 5 revealed steatosis in 3, steatosis and fibrosis in 1, and increase in lipofuchsin pigment in another. Detailed investigation revealed no known metabolic defect, adverse environmental exposure, or alcohol abuse. We postulate that this family represents a unique type of idiopathic familial cirrhosis. The role of steatosis in the pathogenesis of cirrhosis in this family remains unsettled. The HLA haplotype A24, B18, DRW 4 X 7 was found in several family members, but the association of the disease with the HLA system remains to be established.