The Nephrotic Syndrome in Adults with ‘Minimal Change’ Glomerular Lesions

Abstract

We describe 49 patients who developed a nephrotic syndrome after the age of 15 years, and who showed a ‘minimal change’ pattern in their renal biopsies. Patients with sclerosing lesions affecting only part of the glomerulus were excluded, but 39 biopsies showed some minor changes. Glomerulosclerosis affecting whole glomeruli was present in 21 biopsies, and related strongly to increasing age. Focal tubular atrophy and vascular changes were also common but less related to age. Mesangial matrix increase was not age-related, and presumably is a component of the glomerular disease. At onset, these patients showed features differing from those found in nephrotic children with similar histology: the sex incidence of the adults was equal, non-selective differential protein 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 from other adult nephrotics, and renal biopsy remains essential in their management. The subsequent behaviour of the adult patients however, resembled that of the childhood group very closely. Forty four patients were treated with prednisolone for at least eight weeks, and 82 per cent responded with early loss of proteinuria. Of the 36 responding patients, 70 per cent later relapsed, 63 per cent repeatedly. Five corticosteroid-resistant, and 12 corticosteroidintolerant patients were treated with cyclophosphamide: one died after a pulmonary embolus, but 14 lost their proteinuria and 11 remain in remission. Follow-up data were available for all 49 patients for up to 19 years (mean 4.1 years). Nine patients were dead, only one in uraemia but three from probable complications of treatment. Ten still relapse and require corticosteroid treatment, but only one now has persistent symptomless proteinuria. Twenty-nine are well, off all treatment.