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一例罕见的肺出血肾炎综合征合并结节性多动脉炎病例。

A Rare Case of Goodpasture's Syndrome Combined with Polyarteritis Nodosa.

作者信息

Sugimoto Hisashi, Sawa Naoki, Oba Yuki, Kurihara Shigekazu, Sekine Akinari, Inoue Noriko, Mizuno Hiroki, Yamanouchi Masayuki, Hasegawa Eiko, Suwabe Tatsuya, Tanaka Kiho, Kono Kei, Kinowaki Keiichi, Ohashi Kenichi, Yamaguchi Yutaka, Wada Takehiko, Ubara Yoshifumi

机构信息

Nephrology Center and the Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan.

Department of Pathology, Toranomon Hospital Toranomon, Japan.

出版信息

Intern Med. 2025 Aug 1;64(15):2356-2360. doi: 10.2169/internalmedicine.4460-24. Epub 2025 Feb 1.

Abstract

A 75-year-old man with a fever, shoulder pain, and lower leg edema was diagnosed with polymyalgia rheumatica and started on glucocorticoid therapy. Eighteen months later, he was admitted with rapidly progressive renal failure. Glucocorticoid therapy had been discontinued one month prior to admission. Serum anti-glomerular basement membrane antibodies were elevated, and a kidney biopsy revealed fibrinoid necrosis of the medium-sized renal arteries, tubulointerstitial nephritis, and collapsed glomeruli. An immunofluorescence study showed mild immunoglobulin G linear deposition. Polyarteritis nodosa was diagnosed based on the presence of fibrinoid necrosis in the medium-sized renal arteries. Glucocorticoid pulse therapy and plasmapheresis were initiated, but the patient died of alveolar hemorrhaging. This was a rare case of Goodpasture's syndrome with polyarteritis nodosa.

摘要

一名75岁男性,伴有发热、肩部疼痛和小腿水肿,被诊断为风湿性多肌痛并开始接受糖皮质激素治疗。18个月后,他因快速进展的肾衰竭入院。入院前1个月已停用糖皮质激素治疗。血清抗肾小球基底膜抗体升高,肾脏活检显示中-sized肾动脉纤维素样坏死、肾小管间质性肾炎和肾小球塌陷。免疫荧光研究显示轻度免疫球蛋白G线性沉积。根据中-sized肾动脉存在纤维素样坏死诊断为结节性多动脉炎。开始糖皮质激素冲击治疗和血浆置换,但患者死于肺泡出血。这是一例罕见的合并结节性多动脉炎的Goodpasture综合征病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/88c4/12393960/da42d9774b07/1349-7235-64-15-2356-g001.jpg

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