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ANCA-negative glomerulonephritis associated with nonasthmatic Churg–Strauss syndrome

Nature Clinical Practice Nephrology volume 4, pages 568–574 (2008)Cite this article

Abstract

Background A 36-year-old white male with a history of allergic rhinitis and sinusitis presented to the emergency room with abdominal pain and diarrhea. Physical examination revealed fever, hypoxemia and a maculopapular rash. Laboratory tests showed proteinuria, hematuria, leukocytosis, eosinophilia and an elevated erythrocyte sedimentation rate.

Investigations Physical examination, urine and blood analysis and culture, chest radiography, chest and abdominal CT, esophagogastroduodenoscopy, skin and kidney biopsy, serology and renal ultrasound.

Diagnosis Pauci-immune focal necrotizing glomerulonephritis with crescents and interstitial eosinophilia, consistent with Churg–Strauss syndrome.

Management The patient was treated with methylprednisolone 1 g/day intravenously for 3 days. He also received oral clotrimazole and combined oral sulfamethoxazole and trimethoprim for prophylaxis against candidiasis and pneumonia, respectively. He left the hospital against medical advice and was lost to follow-up.

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Figure 1: The renal biopsy reveals a glomerular segment with deeply acidophilic, fibrinoid necrosis surrounded by a small cellular crescent.
Figure 2: High-magnification photomicrograph of the renal biopsy sample showing extensive interstitial inflammation with numerous eosinophils.
Figure 3: Postulated pathogenesis of ANCA-mediated pauci-immune vasculitis.

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Acknowledgements

The views expressed in this article are those of the authors and do not necessarily represent the views of the Department of Veterans Affairs. Charles P Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. AJ Chemmalakuzhy is a Postdoctoral Clinical Fellow in the Division of Nephrology at the University of Texas Southwestern Medical Center, and SS Hedayati is an Assistant Professor of Medicine in the Division of Nephrology at the University of Texas Southwestern Medical Center, XJ Zhou is a Professor of Pathology and Internal Medicine at the University of Texas Southwestern Medical Center, and Attending Physician at the Veterans Affairs North Texas Healthcare System, Dallas, TX, USA.,

    Asha J Chemmalakuzhy, Xin J Zhou & S Susan Hedayati

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  1. Asha J Chemmalakuzhy
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Correspondence to S Susan Hedayati.

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Chemmalakuzhy, A., Zhou, X. & Hedayati, S. ANCA-negative glomerulonephritis associated with nonasthmatic Churg–Strauss syndrome. Nat Rev Nephrol 4, 568–574 (2008). https://doi.org/10.1038/ncpneph0922

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