Ten years follow-up of idiopathic focal and segmental glomerulosclerosis

Sixty-four biopsy proven cases of idiopathic focal and segmental glomerulosclerosis (FSGS) were studied retrospectively for 10 years. The mean age of the study patients was 29.5 years and 81.2% of the patients were males. Edema, hematuria, hypertension and azotemia were the main presenting features. All cases were initially treated with steroids alone. Nineteen cases (29.7%) had complete and 14 patients (21.9%) had partial remission while 31 cases (48.4%) had no response. Twenty non-responsive cases were given cyclophosphamide in addition to steroids and seven (35%) patients had complete remission and a similar number had partial remission. Patients who had azotemia, hypertension, nephrotic range proteinuria and diffuse mesangial hypercellularity in addition to segmental sclerosis on renal histology at presentation were classified as malignant FSGS. There were eight such cases of which two showed partial response to steroids. One patient went into complete remission with steroids and cyclophosphamide while two others showed partial remission. Mean duration before the occurrence of renal death in benign FSGS patients was six years while all malignant FSGS cases had renal death within two years. Differentiating benign and malignant FSGS will help in prognostication of patients with this pathology.