FAMILIAL AUTOIMMUNE HEPATITIS AND C4 DEFICIT

Introduction. - Familial auto-immune hepatitis is unusual. We report a case in which hepatitis was associated with a deficiency in the C4 co mponent of the complement. Exegesis. - Type I auto-immune hepatitis A was diagnosed in a 38-year-old woman presenting with systemic lupus er ythematosus. Her daughter had to undergo a splenectomy for immunologic thrombocytopenic purpura when she was 9 years old. When she turned 13 , she further developed type I autoimmune hepatitis. During follow-up (4 and 8 years, respectively), both patients had a mild deficiency in C4. Conclusion. - C4 deficiency is not only frequently observed in rel atives of patients with auto-immune hepatitis, but also in familial sy stemic lupus. This abnormality may have had a crucial pathogenic role in these Two patients. (C) 1998 Elsevier, Paris.