Up to 80% of all cases of pulmonary-renal syndrome are due to systemic vasculitis associated with antibodies to neutrophil cytoplasm (ANCA). Most patients die within the first year after the onset of the disease but adequate immunosuppressive therapy ensures the 5-year survival rate of 65-75%. We obtained clinical and morphological data for 34 patients with ANCA-associated vasculitis affecting lungs and kidneys treated at S. P. Botkin Hospital in 1994-2012. 16 patients presented with granulomatous polyangiitis (Wegener 's granulomatosis), 11 with microscopic polyangiitis, and 7 with eosinophilicpolyangiitis (Churg-Strauss syndrome). We made the definitive diagnosis in 79% of the cases. Pulmonary lesions were largely in the form offocal, nodular and infiltrative changes, one third of the patients suffered blood spitting/pulmonary hemorrhage and 15% respiratory distress. Most patients had renal dysfunction (16% required emergency hemodialysis), lesions of ENT skin, joints, peripheral nervous system, and eyes. Therapy with corticosteroids and cytostatics increased the total and renal survival rate up to 91 and 82% respectively despite unfavourable prognostic factors in most patients. Duration of observation was 32.5 [0.1;129] months, the survival rate depended on the severity of lung lesions whereas renal problems had no appreciable effect on life prognosis.