In hereditary nephritis angiotensin-converting enzyme inhibition decreases proteinuria and may slow the rate of progression.

The hereditary nephritides are often progressive, resulting in kidney failure and the need for renal replacement therapy. There is no currently known beneficial treatment for these disorders. We observed three patients with hereditary glomerulonephritis with plasma creatinine concentrations ranging from 1.7 to 2.0 mg/dL who were treated with angiotensin-converting enzyme inhibitors (ACEIs) for 3.5 to 6 years. Angiotensin-converting enzyme inhibitor therapy was accompanied by a decrease in the mean arterial pressure (MAP) from 115 +/- 10 mm Hg to 93 +/- 2 mm Hg (+/- SD), a decrease in the mean urinary protein/creatinine ratio from 2,910 +/- 1,720 mg/g to 391 +/- 355 mg/g, and stabilization of the decline of creatinine clearance with time in two of the three patients. Based on this apparent benefit of ACEIs in hereditary nephritis, we suggest that a prospective controlled trial of ACEIs should be undertaken among a large group of such patients. Pending the results of such a study, ACEIs should be considered for the treatment of patients with proteinuric and progressive hereditary nephritis.