The role ANCA and anti-GBM antibodies in pulmonary-renal syndrome due to Wegener's granulomatosis.

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.