Background: Antineutrophil cytoplasmic antibodies (ANCA) have been proven to be useful diagnostic tools in patients with systemic vasculitis and glomerulonephritis. These antibodies exist in two types, a cytoplasmic pattern (cANCA) and a perinuclear pattern (pANCA). The effect of the main ANCA subtypes on renal outcome and its relationship to demographic findings and clinical features of patients with ANCA-associated glomerulonephritis has not been adequately studied. Patients and Method:In this prospective study, we compared the clinical features at presentation and the renal outcome after 1 year of follow-up between two groups of patients with cANCA (n=22) and pANCA (n=29) consecutively encountered over a one-year period. Result: At presentation, rapidly progressive glomerulonephritis (RPGN), and after 1 year of follow-up, end-stage renal disease (ESRD) were seen more commonly in patients with pANCA than cases with cANCA (P=0.001 and P=0.004, respectively). Seropositivity for cANCA was more common in male and pANCA in female patients (P=0.005). Occurrence of the pulmonary-renal syndrome or extra-renal manifestations, such as sinusitis and skin rash, did not differ significantly among the two groups of patients with cANCA and pANCA. Conclusion: Patients with pANCA present more frequently with RPGN, leading to a poorer renal survival compared to cases with cANCA. RPGN and pANCA are more common in females.