@article{oai:kanazawa-u.repo.nii.ac.jp:00013964,
 author = {山崎, 雅英},
 issue = {6},
 journal = {日本臨床免疫学会会誌 = Japanese journal of clinical immunology},
 month = {Dec},
 note = {反復性血栓症と不育症を特徴とする自己免疫性血栓性疾患である抗リン脂質抗体症候群の中に，微小血栓により短期間に多臓器不全をきたす予後不良の一群があり近年注目されている．このような疾患群を「劇症型抗リン脂質抗体症候群（CAPS）」という．CAPSは感染症や抗血栓療法の変更，手術（抜歯などの小手術を含む）を契機に，SLEや原発性抗リン脂質抗体症候群症例に多く発症し，脳血管系・呼吸器系・腎臓・皮膚などのほか，全身のすべての臓器に微小血栓をきたす．確立した治療法は無いが，強力な抗凝固療法と大量ステロイド療法がおこなわれるほか，血漿交換も併用されることが多い．我々の経験では，抗リン脂質抗体や抗二重鎖DNA抗体（抗ds-DNA抗体），補体などを選択的に吸着する血漿吸着療法を血漿交換の代わりに用いることにより良好な成績が得られている．血漿吸着療法は血漿交換と比較して新鮮凍結血漿などの血液製剤の補充が不要であり，輸血関連合併症もないことからCAPSを含む抗リン脂質抗体症候群に対し考慮すべき治療法の1つと考えられる． Antiphospholipid syndrome (APS) is well known as an autoimmune thrombotic syndrome with recurrent thromboses. In APS, thromboses occurs both artery and vein, and from large to micro vessels. In contrast, so called catastrophic antiphospholipid syndrome, CAPS, develops multiple thromboses at microvessels mainly within a few weeks and induces to poor prognosis. CAPS often occurs in patients with SLE or primary APS after a change of antithrombotic therapy, infection, and operation. Treatments for CAPS have not established although plasma exchange is carried out usually as well as intensive anticoagulation and immunosuppressive therapy. We treated with immunoadsorption plasmapheresis (IAPP) for 5 CAPS patients and they improved their clinical symptoms and ameliorated their titers of antiphospholipid antibodies. IAPP could be an useful treatment skill for CAPS and we have started prospective study.},
 pages = {357--364},
 title = {劇症型抗リン脂質抗体症候群},
 volume = {28},
 year = {2005}
}