Ayuzawa Nobuhiro, Ubara Yoshifumi, Keiichi Sumida, Masayuki Yamanouchi, Hasegawa Eiko, Hiramatsu Eriko, Hayami Noriko, Suwabe Tatsuya, Hoshino Junichi, Sawa Naoki, Kawabata Masateru, Ohashi Kenichi, Takaichi Kennmei
Nephrology Center, Toranomon Hospital, Japan.
Intern Med. 2012;51(10):1233-8. doi: 10.2169/internalmedicine.51.6074. Epub 2012 May 15.
A 68-year-old Japanese woman with asthma of recent onset and a long history of membranous glomerulonephropathy (MN) was admitted because of multifocal pulmonary infiltrates, marked eosinophilia, mild renal dysfunction, a rash on her feet, and right median nerve paralysis. Although MPO- and PR3-ANCA were negative, skin biopsy demonstrated leukocytoclastic vasculitis and Churg-Strauss Syndrome (CSS) was diagnosed. She also had salivary gland swelling and a high serum IgG4 level. Renal biopsy revealed MN with eosinophil-rich tubulointerstitial nephropathy. Her symptoms resolved after the start of corticosteroid therapy. The present case shows that ANCA-negative CSS can have a clinical condition similar to IgG4-related kidney disease.
一名68岁的日本女性，近期患哮喘，有长期膜性肾小球肾病（MN）病史，因多灶性肺浸润、明显嗜酸性粒细胞增多、轻度肾功能不全、足部皮疹及右正中神经麻痹入院。尽管髓过氧化物酶（MPO）-抗中性粒细胞胞浆抗体（ANCA）和蛋白酶3（PR3）-ANCA均为阴性，但皮肤活检显示白细胞破碎性血管炎，诊断为变应性肉芽肿性血管炎（CSS）。她还出现唾液腺肿大及血清IgG4水平升高。肾活检显示MN合并富含嗜酸性粒细胞的肾小管间质性肾病。开始使用糖皮质激素治疗后，她的症状得到缓解。本病例表明，ANCA阴性的CSS可具有与IgG4相关性肾病相似的临床情况。
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