title: [Familial autoimmune hepatitis and C4 deficiency].
abstract: BACKGROUND
  Familial auto-immune hepatitis is unusual. We report a case in which hepatitis was associated with a deficiency in the C4 component of the complement.
  
  
  METHODS
  Type 1 auto-immune hepatitis A was diagnosed in a 38-year-old woman presenting with systemic lupus erythematosus. 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 1 autoimmune hepatitis. During follow-up (4 and 8 years, respectively), both patients had a mild deficiency in C4.
  
  
  CONCLUSIONS
  C4 deficiency is not only frequently observed in relatives of patients with auto-immune hepatitis, but also in familial systemic lupus. This abnormality may have had a crucial pathogenic role in these two patients.
authors.affiliation: Service de médecine interne et pathologie vasculaire, hôpital Saint-André, Bordeaux, France.
authors.forename: J
authors.initials: J
authors.lastname: Constans
authors.forename: P
authors.initials: P
authors.lastname: Bernard
authors.forename: P
authors.initials: P
authors.lastname: Bioulac-Sage
authors.forename: D
authors.initials: D
authors.lastname: Barcat
authors.forename: C
authors.initials: C
authors.lastname: Conri
mesh_terms: D000293:Adolescent; D000328:Adult; D003181:Complement C4 / Q000172:deficiency*; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006506:Hepatitis A / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D011696:Purpura, Thrombocytopenic / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D013997:Time Factors
chemical_list: D003181:Complement C4
vernacular_title: Hépatite auto-immune familiale et déficit en C4.