The Nephrotic Syndrome in Adults with 'Minimal Change' Glomerular Lesions

We describe 49 patients who developed a nephrotic syndrome afterthe age of 15 years, and who showed a ‘minimal change’pattern in their renal biopsies. Patients with sclerosing lesionsaffecting only part of the glomerulus were excluded, but 39biopsies showed some minor changes. Glomerulosclerosis affectingwhole glomeruli was present in 21 biopsies, and related stronglyto increasing age. Focal tubular atrophy and vascular changeswere also common but less related to age. Mesangial matrix increasewas not age-related, and presumably is a component of the glomerulardisease. At onset, these patients showed features differing from thosefound in nephrotic children with similar histology: the sexincidence of the adults was equal, non-selective differentialprotein clearances (51 per cent) hypertension (31 per cent)and diminished renal function (70 per cent) were all more common.No clinical tests distinguished these patients clearly fromother adult nephrotics, and renal biopsy remains essential intheir management. The subsequent behaviour of the adult patientshowever, resembled that of the childhood group very closely.Forty four patients were treated with prednisolone for at leasteight weeks, and 82 per cent responded with early loss of proteinuria.Of the 36 responding patients, 70 per cent later relapsed, 63per cent repeatedly. Five corticosteroid-resistant, and 12 corticosteroidintolerantpatients were treated with cyclophosphamide: one died aftera pulmonary embolus, but 14 lost their proteinuria and 11 remainin remission. Follow-up data were available for all 49 patients for up to19 years (mean 4.1 years). Nine patients were dead, only onein uraemia but three from probable complications of treatment.Ten still relapse and require corticosteroid treatment, butonly one now has persistent symptomless proteinuria. Twenty-nineare well, off all treatment.