CASE REPORTS   

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

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.