In 1994, we reported a 3.4 +/- 0.8 year follow-up of the eight patients who experienced remission of nephrotic syndrome during the Collaborative Study Group-sponsored, multicenter trial of captopril therapy in patients with type 1 diabetes with nephropathy (Captopril Study). Of the 409 patients randomized to treatment on the Captopril Study, 108 had nephrotic syndrome (24-hour proteinuria >/= 3.5 g of protein) at baseline. Of these 108 patients, 8 experienced remission of nephrotic syndrome (proteinuria </= 1.0 g/24 h of protein). Remission was significantly associated with captopril therapy and control of systolic blood pressure. The present study describes the status of these eight patients during a follow-up of 7.7 +/- 0.3 years. Since our previous report, one patient has been lost to follow-up and one patient progressed to end-stage renal disease (ESRD) 3.7 years after completion of the Captopril Study. The remaining six patients remain in remission of nephrotic syndrome (mean 24-hour proteinuria, 1.03 +/- 0.3 g of protein) and have stable serum creatinine levels (mean, 1.58 +/- 0.3 mg/dL) and body weights (mean, 69.8 +/- 5.3 kg). Of the six patients, one has discontinued angiotensin-converting enzyme inhibitor (ACEi) therapy because of hypotension. Excluding the patient who progressed to ESRD, the current mean systolic blood pressure is 135 +/- 6 mm Hg and mean diastolic blood pressure is 78 +/- 4 mm Hg. We conclude that long-term remission of nephrotic syndrome and preservation of renal function is achievable in some patients with type 1 diabetes. Control of blood pressure and ACEi therapy appear to be important in achieving long-term remission.