BACKGROUND: Anti-neutrophil cytoplasmic antibodies (ANCA) define pathogenetically related group of renal vasculitides and glomerulonephritides mostly with serious prognosis. If unrecognized, these life-threatening diseases may cause loss of independent renal function and other dangerous extrarenal complications (e. g. pulmonary haemorrhage). We concentrated on the diagnosis, treatment and log-term follow-up of these patients. METHODS AND RESULTS: Renal biopsy was performed in 46 ANCA-positive patients. Age and sex distribution, type of ANCA, organ involvement, renal biopsy findings, renal function and effect of therapy were analyzed in these patients. Twenty three patients suffered from renal vasculitis, most commonly Wegener's granulomatosis (14 patients) and microscopic polyarthritis (7 patients). IgA nephropathy (7 patients) and idiopathic necrotizing/crescentic glomerulonephritis (8 patients) prevailed in patients with limited renal involvement. Renal morphology and function was most seriously impaired in patients with Wegener's granulomatosis. Immunosuppressive treatment was able to control the activity of the disease with the negativization of ANCA and improvement or stabilization of renal function in more than 90% of patients.CONCLUSIONS. ANCA-positive renal vasculitis and glomerulonephritis is relatively common. Clinical signs of extrarenal involvement are present in about 50% of patients with ANCA-positive glomerulonephritis. Rapidly introduced immunosuppressive treatment effectively controls renal and extrarenal manifestations of the disease.

1 interní klinika 1 LF UK a VFN Praha