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Churg-Strauss Syndrome with a Clinical Condition Similar to IgG4-Related Kidney Disease: A Case Report

2012; The Japanese Society of Internal Medicine; Volume: 51; Issue: 10 Linguagem: Inglês

10.2169/internalmedicine.51.6074

1349-7235

Nobuhiro Ayuzawa, Yoshifumi Ubara, Keiichi Sumida, Masayuki Yamanouchi, Eiko Hasegawa, Eriko Hiramatsu, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Masateru Kawabata, Kenichi Ohashi, Kennmei Takaichi,

Urticaria and Related Conditions

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.