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Rev Med Interne. 1998 Oct;19(10):731-3.

[Familial autoimmune hepatitis and C4 deficiency].

[Article in French]

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  • 1Service de médecine interne et pathologie vasculaire, hôpital Saint-André, Bordeaux, France.



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.


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.


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.

[PubMed - indexed for MEDLINE]
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