Rapidly progressive glomerulonephritis (RPGN) is rarely associated with macroscopic polyarteritis nodosa (PAN), as seen in this series of 7 out of 235 patients. The clinical symptoms of PAN were as follows: myalgias 6; fever 1; arthralgias and mononeuropathy multiplex 5; cutaneous vasculitis 3; arterial hypertension 4, 3 of which were malignant. The mean proteinuria was 2.7 g/24 h; creatininemia 458 microM/l; microscopic hematuria was present in 4 of the 7 patients; only 1 patient was anuric. Markers of hepatitis B virus were absent in all cases. Arteriography revealed microaneurysms and renal infarcts in 6 patients and distal arterial stenosis in one. Renal biopsies from all 7 patients demonstrated extracapillary glomerulonephritis, which was associated with tubulointerstitial fibrosis in one. Necrotizing vasculitis lesions were associated in 5 cases. Immunofluorescence was positive in 5 cases. The association of RPGN and PAN exists and may be underestimated due to the lack of systematic angiographic examinations during RPGN and renal biopsies in PAN patients with renal involvement.