Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Churg-Strauss Syndrome with a Clinical Condition Similar to IgG4-Related Kidney Disease: A Case Report
Nobuhiro Ayuzawa, Yoshifumi Ubara, Sumida Keiichi, Yamanouchi Masayuki, Eiko Hasegawa, Eriko Hiramatsu, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Masateru Kawabata, Kenichi Ohashi, Kennmei Takaichi
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Keywords: IgG4-related kidney disease, Churg-Strauss syndrome, ANCA negative, membranous nephropathy, tubulointerstitial nephritis
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2012 Volume 51 Issue 10 Pages 1233-1238

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Abstract

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.

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© 2012 by The Japanese Society of Internal Medicine
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