Familial auto-immune hepatitis and C4 deficit.

被引：4

作者：

Constans, J ^{[1
]}

Bernard, P ^{[1
]}

Bioulac-Sage, P ^{[1
]}

Barcat, D ^{[1
]}

Conri, C ^{[1
]}

机构：

[1] Hop St Andre, Serv Med Interne & Pathol Vasc, F-33075 Bordeaux, France

来源：

REVUE DE MEDECINE INTERNE | 1998年 / 19卷 / 10期

关键词：

auto-immune hepatitis; systemic lupus erythematosus; deficit in complement;

D O I：

10.1016/S0248-8663(98)80709-1

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Introduction. - 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. Exegesis. - Type I 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 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 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. (C) 1998 Elsevier, Paris.

引用

页码：731 / 733

页数：3

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