title: The role ANCA and anti-GBM antibodies in pulmonary-renal syndrome due to Wegener's granulomatosis.
abstract: Pulmonary-renal syndrome (PRS) is defined as a diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. We present a retrospective study of 22 consecutive patients with Wegener's granulomatosis (WG). Logistic regression analysis and a Wilcoxon test were included in the statistics. Survival time death risk were assessed using the Kaplan-Meier estimator and the Cox proportional hazard model. At recognition, the median Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) was 30.0 (23.0-32.5), PO2 on air was 5.8+/-0.5 kPa, creatinine level was 7.2+/-1.4 mg/dl. Fifteen patients were PR3 positive, among them 4 patients were also positive for anti-glomerular basement membrane antibodies (anti-GBM). Renal biopsy was performed in 16 patients. Histological examination reviled segmental necrotizing crescentic GN in 15 patients. Thirteen patients were initially dialysis-dependent, and 7 required ventilatory support. All patients were treated with methylprednisolone and cyclophosphamide (pulses). The patients were followed up for 24+/-8 months. Of the survivors, 55% and 31% were alive after 1 and 2 years. Early recognition and proper treatment may improve outcome in PRS.
authors.affiliation: Systemic Vasculitis Outpatient Clinic of the Department of Family Medicine, Internal and Metabolic Diseases, Nephrology Unit, Warsaw Medical University, Warsaw, Poland. kzycinska@poczta.fm
authors.forename: K
authors.initials: K
authors.lastname: Zycinska
authors.forename: K A
authors.initials: KA
authors.lastname: Wardyn
authors.forename: T M
authors.initials: TM
authors.lastname: Zielonka
authors.forename: M
authors.initials: M
authors.lastname: Otto
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000502:physiology*; D001323:Autoantibodies / Q000276:immunology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007674:Kidney Diseases / Q000209:etiology* / Q000276:immunology*; D008171:Lung Diseases / Q000209:etiology* / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000097:blood; D009195:Peroxidase / Q000097:blood; D006435:Renal Dialysis; D016019:Survival Analysis
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D009195:Peroxidase; D053146:Myeloblastin