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A typical Wegeners granulomatosis -- but not pauci-immune!

Abstract
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.
AuthorsL Joerg, A Kerstin, B Niko, A Dominik, K Martin, J Latus, K Amann, N Braun, M D Alscher, M Kimmel
JournalMinerva urologica e nefrologica = The Italian journal of urology and nephrology (Minerva Urol Nefrol) Vol. 64 Issue 2 Pg. 149-52 (Jun 2012) ISSN: 0393-2249 [Print] Italy
PMID22617309 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Antibodies, Antineutrophil Cytoplasmic
  • Biomarkers
  • Glucocorticoids
  • Immunoglobulin A
  • Immunologic Factors
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisolone
Topics
  • Antibodies, Antineutrophil Cytoplasmic (blood)
  • Biomarkers (blood)
  • Cyclophosphamide (therapeutic use)
  • Drug Therapy, Combination
  • Follow-Up Studies
  • Glomerulonephritis, IGA (blood, diagnosis, drug therapy, immunology)
  • Glucocorticoids (therapeutic use)
  • Granulomatosis with Polyangiitis (blood, diagnosis, drug therapy, immunology)
  • Humans
  • Immunoglobulin A (blood)
  • Immunologic Factors (blood)
  • Immunosuppressive Agents (therapeutic use)
  • Male
  • Middle Aged
  • Prednisolone (therapeutic use)
  • Tomography, X-Ray Computed
  • Treatment Outcome

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