Background. Immunoglobulin A nephropathy (IgAN) is the most common cause of chronic renal failure among primary glomerulonephritis patients. The best treatment for IgAN remains poorly defined. We planned a longterm, prospective, open-label, multicentre, centrally randomized controlled trial to assess whether the combination of prednisone and ramipril was more effective than ramipril alone in patients with proteinuric IgAN. Methods. Ninety-seven biopsy-proven IgAN patients with moderate histologic lesions, 24-h proteinuria ≥1.0 g and estimated glomerular filtration rate (eGFR) ≥ 50 ml/min/ 1.73 m2 were randomly allocated to receive a 6-month course of oral prednisone plus ramipril (combination therapy group) or ramipril alone (monotherapy group) for the total duration of follow-up. The primary outcome was the progression of renal disease defined as the combination of doubling of baseline serum creatinine or end-stage kidney disease (ESKD). The secondary outcomes were the rate of renal function decline defined as the eGFR slope over time, and the reduction of 24-h proteinuria. Results. After a follow-up of up to 96 months, 13/49 (26.5%) patients in the monotherapy group reached the primary outcome compared with 2/48 (4.2%) in the combination therapy group. The Kaplan–Meier analysis showed a significantly higher probability of not reaching the combined outcome in the combination therapy group than in the monotherapy group (85.2% versus 52.1%; log-rank test P=0.003). In the multivariate analysis, baseline serum creatinine and 24-h proteinuria were independent predictors of the risk of primary outcome; treatment with prednisone plus ramipril significantly reduced the risk of renal disease progression (hazard ratio 0.13; 95% confidence interval 0.03–0.61; P = 0.01). The mean rate of eGFR decline was higher in the monotherapy group than in the combination therapy group (−6.17 ± 13.3 versus −0.56 ± 7.62 ml/min/ 1.73 m2/year; P = 0.013). Moreover, the combined treatment reduced 24-h proteinuria more than ramipril alone during the first 2 years. Conclusions. Our results suggest that the combination of corticosteroids and ramiprilmay provide additional benefits compared with ramipril alone in preventing the progression of renal disease in proteinuric IgAN patients in the longterm follow-up.