title: 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.
authors.affiliation: Department of Internal Medicine, Robert-Bosch Hospital, Stuttgart, Germany. joerg.latus@rbk.de
authors.forename: L
authors.initials: L
authors.lastname: Joerg
authors.forename: A
authors.initials: A
authors.lastname: Kerstin
authors.forename: B
authors.initials: B
authors.lastname: Niko
authors.forename: A
authors.initials: A
authors.lastname: Dominik
authors.forename: K
authors.initials: K
authors.lastname: Martin
authors.forename: J
authors.initials: J
authors.lastname: Latus
authors.forename: K
authors.initials: K
authors.lastname: Amann
authors.forename: N
authors.initials: N
authors.lastname: Braun
authors.forename: M D
authors.initials: MD
authors.lastname: Alscher
authors.forename: M
authors.initials: M
authors.lastname: Kimmel
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D005922:Glomerulonephritis, IGA / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007155:Immunologic Factors / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D005938:Glucocorticoids; D007070:Immunoglobulin A; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone