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症例は45歳男性.4年前に全身性エリテマトーデスと診断され,プレドニゾロン(PSL)内服を開始.以降,PSLを漸減しながら観察されていた.PSLを10mg隔日投与から7.5mg隔日投与に漸減された約1カ月後より腹痛,下痢,発熱が出現し緊急入院.活動性SLEが判明したため腸管の血管炎を疑われPSLの増量が試みられた.しかし,症状は改善せず遂には腸管穿孔による腹膜炎を併発し,緊急手術となった.開腹所見では上腸間膜動脈(SMA)起始部の血栓による狭窄と小腸の広範な壊死が認められた.病理組織検査では腸間膜の細小動静脈に血管炎の像も認められたがSMAには特異的な所見はなく,術前の血清学的検査でaCL-β2GPI抗体陽性が判明したことより,この症例をSLEに伴う抗リン脂質抗体症候群と診断した.
A 45-year-old man, who had been followed up as case of Systemic Lupus Erythematosus (SLE) for 4 years. was admitted to our hospital because of fever, abdominal pain and diarrhea. He had been treated with corticoster-oid therapy and the close of predonisolone (PSL) had been decreased from 10mg to 7. 5 mg every other day bout one month before admission. Laboratory findings owed that the SLE was in the active stage, we suspect-ed vasculitis of the small or large intestine. Conserva-tive treatment with increasing PSL close up to 60mg/ day, however, was not effective, and laparotomy was performed because CT examination confirmed perfora-tion of the intestine. Thrombosis of the superior mesentric artery (SMA) and necroses in the entire small intestine were found. Although vasculitis was detected in small vessels within the mesenterium, there was neither arteriosclerosis nor vasculitis in the SMA wall.We found that anti-cardiolipin β2 glycoprotein I com-plex antibody was positive, so we diagnosed this case as anti-cardiolipin-antibody syndrome with SLE.
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