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Minerva Urologica e Nefrologica 2012 June;64(2):149-52

Copyright © 2012 EDIZIONI MINERVA MEDICA

language: English

A typical Wegeners granulomatosis - but not pauci-immune!

Joerg L. 1, Kerstin A. 2, Niko B. 1, Dominik A. M. 1, Martin K. 1, Latus J. 1, Amann K. 2, Braun N. 1, Alscher M. D. 1, Kimmel M. 1

1 Division of Nephrology, Department of Internal Medicine, Robert-Bosch Hospital, Stuttgart, Germany; 2 Department of Pathology, University of Erlangen-Nuernberg, Erlangen-Nuernberg, Germany


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We present a the case of 58-year old man who was admitted to hospital with typical clinical features (bloody nasal discharge, arthralgia, acute kidney injury with a nephritic syndrome) consisting with Wegeners granulomatosis (WG). CT-scan showed pulmonary nodules and antineutrophil cytoplasmatic antibodies (ANCA) were elevated. A kidney biopsy showed a crescentic glomerulonephritis, but not pauci-immune-immune with a histopathological staining of a mesangioproliferative IgA-glomerulonephritis. The patient was put on prednisolone and i.v. cyclophosphamid (CYCLOPS-protocol (1). The anti-proteinase-3 antibody titer decreased and the CT-scan showed decreased activity of Wegener’s granulomatosis (BVAS 26 dropped to 2) and the patient`s serum creatinine level was stable. The exact nosological relation of mesangial IgA-nephropathy to WG is still unclear. This case underlines that knowledge of renal histology is essential in the management of patients with renal disease, especially in patients with hematuria and/or proeinuria with positive ANCA.

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