Cerebral thrombotic microangiopathy and antiphospholipid antibodies in Aicardi-Goutieres syndrome report of two sisters

被引:37
作者
Rasmussen, M [1 ]
Skullerud, K
Bakke, SJ
Lebon, P
Jahnsen, FL
机构
[1] Natl Hosp Norway, Dept Pediat, N-0027 Oslo, Norway
[2] Univ Oslo, Rikshosp, Dept Pathol, N-0027 Oslo, Norway
[3] Univ Oslo, Rikshosp, Inst Pathol, N-0027 Oslo, Norway
[4] Natl Hosp Norway, Dept Radiol, N-0027 Oslo, Norway
[5] Univ Paris 05, Hop St Vincent de Paul, Serv Virol, Paris, France
关键词
Aicardi-Goutieres syndrome; thrombotic microangiopathy; antiphospholipid antibodies; systemic lupus erythematosus; interferon;
D O I
10.1055/s-2004-830532
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Cerebral thrombotic microangiopathy was found at autopsy in one of two sisters with Aicardi-Goutieres syndrome, whereas the other revealed increased serum levels of anticardiolipin IgG antibodies (measured only in the living sister); both typical features of systemic lupus erythematosus. These findings add support to the suggestion that Aicardi-Goutieres syndrome and systemic lupus erythematosus are closely related disorders in which dysregulated production of interferon-alpha might play a crucial role.
引用
收藏
页码:40 / 44
页数:5
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