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増大特集 Close Encounters―臨床神経学と臨床免疫学の遭遇と未来
文献概要
はじめに
抗リン脂質抗体症候群（antiphospholipid syndrome：APS)1)は，1963年にBowieら2)が全身性エリテマトーデス（systemic lupus erythematosus：SLE）における凝固抑制因子の存在を発見した当時はSLEに合併する疾患とされ，ループスアンチコアグラント（lupus anticoagulant：LA）などの抗リン脂質抗体が陽性の血栓症あるいは習慣性流産の原因となるものと定義された。その後1983年，Harrisら3)によって，SLEに合併する脳梗塞において抗カルジオリピン抗体が高頻度に出現していることが明らかにされ，1985年，Hughesら4)によって，独立した疾患概念として提唱された。
APSは免疫学的な機序を基盤として起こる血栓症として重要で，特に若年性脳梗塞の発症5)や，多臓器の動・静脈血栓症，習慣性流産などに関与し，多彩な臨床像を呈する後天的な凝固異常症である。1998年，札幌で開かれたAPSのシンポジウムでその分類診断基準が提唱され6)，2006年に札幌クライテリア・シドニー改変7)としてAPSにおける診断基準が改訂され（Table1），本症候群と脳血管障害の関連についての研究も進められている。最近では，高血圧症の頻度が高く，中枢神経症状が主体となる特殊型として，劇症型（catastrophic）APS8)も注目されている。
抗リン脂質抗体症候群（antiphospholipid syndrome：APS)1)は，1963年にBowieら2)が全身性エリテマトーデス（systemic lupus erythematosus：SLE）における凝固抑制因子の存在を発見した当時はSLEに合併する疾患とされ，ループスアンチコアグラント（lupus anticoagulant：LA）などの抗リン脂質抗体が陽性の血栓症あるいは習慣性流産の原因となるものと定義された。その後1983年，Harrisら3)によって，SLEに合併する脳梗塞において抗カルジオリピン抗体が高頻度に出現していることが明らかにされ，1985年，Hughesら4)によって，独立した疾患概念として提唱された。
APSは免疫学的な機序を基盤として起こる血栓症として重要で，特に若年性脳梗塞の発症5)や，多臓器の動・静脈血栓症，習慣性流産などに関与し，多彩な臨床像を呈する後天的な凝固異常症である。1998年，札幌で開かれたAPSのシンポジウムでその分類診断基準が提唱され6)，2006年に札幌クライテリア・シドニー改変7)としてAPSにおける診断基準が改訂され（Table1），本症候群と脳血管障害の関連についての研究も進められている。最近では，高血圧症の頻度が高く，中枢神経症状が主体となる特殊型として，劇症型（catastrophic）APS8)も注目されている。
参考文献
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