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発症に溶連菌感染の関与が示唆されたANCA陰性顕微鏡的多発血管炎の1例 ANCA-negative microscopic polyangiitis associated with streptococcal infection in a 12-year-old boy ハッショウ ニ ヨウレンキン カンセン ノ カンヨ ガ シサ サレタ エイエヌシーエイ インセイ ケンビキョウテキ タハツ ケッカンエン ノ イチレイ 00612376 田中, 悦子 タナカ, エツコ Tanaka, Etsuko 田中 タナカ Tanaka 悦子 エツコ Etsuko 宮崎大学 University of Miyazaki 今村, 秀明 イマムラ, ヒデアキ Imamura, Hideaki 今村 イマムラ Imamura 秀明 ヒデアキ Hideaki 80315366 此元, 隆雄 コノモト, タカオ Konomoto, Takao 此元 コノモト Konomoto 隆雄 タカオ Takao 宮崎大学 University of Miyazaki Nunoi, Hiroyuki 布井, 博幸 ヌノイ, ヒロユキ Nunoi 布井 ヌノイ Hiroyuki 博幸 ヒロユキ 久野, 敏 ヒサノ, サトシ 尾田, 高志 オダ, タカシ 00612376 田中, 悦子 タナカ, エツコ Tanaka, Etsuko 田中 タナカ Tanaka 悦子 エツコ Etsuko 宮崎大学 University of Miyazaki 今村, 秀明 イマムラ, ヒデアキ Imamura, Hideaki 今村 イマムラ Imamura 秀明 ヒデアキ Hideaki Konomoto, Takao Nunoi, Hiroyuki 布井, 博幸 ヌノイ, ヒロユキ Nunoi 布井 ヌノイ Hiroyuki 博幸 ヒロユキ Hisano, Satoshi Hisano Satoshi Oda, Takashi 顕微鏡的多発血管炎, pauci-immune型糸球体腎炎, ANCA, 溶連菌感染症 NAPlr, Microscopic polyangiitis, pauci-immune glomerulonephritis, antineutrophil cytoplasmic antibody, streptococcal infection 今回われわれは,発症に溶連菌感染の関与が示唆された若年発症のANCA陰性顕微鏡的多発血管炎 （MPA） の症例を経験した。症例は12歳男児。発熱,腹痛,皮疹および急性腎不全を呈し,血液浄化療法を要した。膵炎や関節炎などを合併し,多臓器にわたる症状から血管炎症候群が疑われた。皮膚生検は白血球破砕性血管炎,腎生検では半月体形成を伴わないpauci-immune型糸球体腎炎で細動脈にフィブリノイド壊死性血管炎を認めた。MPO-ANCA,PR3-ANCAはともに陰性でありANCA陰性のMPAと診断し,ステロイドによる治療で症状は速やかに軽快した。ASKが高値であり溶連菌感染の関与が疑われ,腎糸球体では溶連菌の腎炎惹起抗原であるNAPlrの染色が陽性であった。これは,溶連菌感染が発症の契機となったことを示すものであり,ANCA陰性MPAの発症機序を検討する上で示唆に富む症例であった。 Microscopic polyangiitis is a type of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis characterized by inflammation of small vessels. Although myeloperoxidase (MPO)-ANCA is usually positive in microscopic polyangiitis, we report the first case of ANCA-negative microscopic polyangiitis associated with streptococcal infection in a 12-year-old boy. He was admitted to our hospital for fever, skin rash, abdominal pain, nephrotic range proteinuria, gross hematuria and renal insufficiency. Subsequent oliguric renal failure, acute pancreatitis and arthritis were observed, and continuous hemodaiafiltration was initiated. He was diagnosed with vasculitis syndrome due to multiple organ symptoms. Laboratory findings revealed negative results for MPO-ANCA and proteinase 3 (PR3)-ANCA. Elevation of antistreptokinase (ASK) titer was observed, but there was no hypocomplementemia. Skin biopsies showed leukocytoclastic vasculitis without IgA and other immunoglobulin deposition. Renal histopathology revealed diffuse endocapillary hypercellularity and mesangial proliferation with mesangiolysis. Crescentic formation was not observed. Severe inflammatory cell infiltration was observed in the entire interstitium with tubulitis. Small-sized vessels, such as afferent arterioles, revealed fibrinoid necrotic angitis with swelling of endothelium. Immunofluorescence studies revealed only a few C3 deposits. Nephritis-associated plasmin receptor (NAPlr), nephritogenic streptococcal antigen, and plasmin activity were seen in the renal biopsy specimens. A diagnosis of ANCAs-negative microscopic polyangiitis with pauci-immune rapidly progressive glomerulonephritis was made and subsequent steroid therapy was initiated which proved to be very effective. Immediate improvement of clinical symptoms and abnormal laboratory findings were seen with complete recovery of renal function. We report the first case of ANCAs negative MPA accompanied with pauci-immune rapidly progressive glomerulonephritis which may have been caused by streptococcal infection due to elevated ASK titers and positive NAPlr and plasmin activity in renal specimens. 出版社版へのリンク:https://www.jstage.jst.go.jp/browse/jjpn/-char/ja/ 日本小児腎臓病学会 The Japanese Society for Pediatric Nephrology 2011-04 jpn journal article VoR http://hdl.handle.net/10458/3850 https://miyazaki-u.repo.nii.ac.jp/records/1588 日本小児腎臓病学会雑誌 Nihon Shoni Jinzobyo Gakkai Zasshi 24 1 96 102 https://miyazaki-u.repo.nii.ac.jp/record/1588/files/1342tanaka.pdf application/pdf 1.9 MB 2020-06-21