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患者は慢性関節リウマチで治療中の72歳の女性で,食思不振のため入院中.上腹部痛が出現し,腹部CTにて,Free air像と著明な腹水がみられ,さらにショック状態を呈した.消化管穿孔による汎発性腹膜炎と診断,開腹手術を施行した.上部空腸に穿孔を認め,空腸部分切除術を施行した.術後,全身状態は徐々に悪化し,第6病日に多臓器不全のため死亡した.血清アミロイドA蛋白(SAA)の著明な上昇と,組織学的に切除小腸の粘膜筋板と血管平滑筋などにアミロイド沈着があった.小腸憩室に穿孔を認め,慢性関節リウマチに続発したアミロイドーシスの消化管穿孔と診断した.アミロイドーシスによる消化管穿孔はまれであるが,慢性関節リウマチの増加に伴い,今後増加することが予想される.慢性関節リウマチの既往のある急性腹症では,消化管アミロイドーシスの鑑別を念頭におく必要があると考えられた.
We report a rare case of small bowel perforation in secondary amyloidosis in a 72-year-old Japanese woman under going treatment for rheumatoid arthritis. The patient developed severe abdominal pain during hospitalization for appetite loss. C-reactive protein was elevated. Abdominal computed tomography (CT) showed free air over the liver and ascites and intestinal dilation, necessitating emergency surgery for diffuse peritonitis secondary to 2 jejunal perforations. We conducted partial resection and reconstructive end-to-end jejunoje-junostomy. Despite post-operative intensive care, her general condition failed to improve and she died of multiple organ failure on postoperative day 6. Postmortem diagnosis was gastrointestinal amyloidosis secondary to rheumatoid arthritis based on histological findings such as amyloid deposits within the muscularis mucosae layer and vascular smooth muscle and perforation in small bowel diverticula, and a marked increase in serum amyloid A (SAA) protein. Although gastrointestinal diverticula and perforation due to amyloidosis is very rare-this is, to our knowledge, the first such report in Japan-we assume the incidence will increase together with recent increases in rheumatoid arthritis. The prognosis of gastrointestinal perforation due to amyloidosis is dismal. Gastrointestinal amyloidosis should be considered a probable diagnosis in patients with acute abdomen and a history of rheumatoid arthritis.
