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In this retrospective study we report the outcome of 59 adults with idiopathic focal-segmental glomerulosclerosis (FSGS) and nephrotic syndrome (NS) treated with corticosteroids and/or immunosuppressive drugs. Twenty-seven patients were initially treated with corticosteroids alone for 9.3 +/- 8 months; nineteen patients received corticosteroids and immunosuppressive agents associated or every other month for 5.5 +/- 4 months; thirteen patients received either azathioprine or cyclophosphamide alone for 25 +/- 27 months. At the end of a mean follow-up of 75 +/- 51 months, 35 patients (60%) were in complete (CR) or partial (PR) remission, 6 (10%) were stable and 18 (30%) had either chronic renal failure (CRF) or end-stage renal failure (ESRF). Out of 36 patients (61%) initially responsive to therapy, 30 (83%) obtained CR, 4 (11%) PR, one (2.7%) developed CRF and one (2.7%) ESRF. Only 10 of the responders (28%) attained remission within 8 weeks of treatment. Out of the 23 (39%) patients originally resistant to therapy, only one (4%) had CR, 6 (26%) remained unchanged, 6 (26%) developed CRF and 10 (43%) ESRF. The presence at initial renal biopsy of interstitial fibrosis was the only feature which could predict a poor renal outcome. These data show that prolonged treatment of FSGS can obtain sustained remission of the disease and improved renal survival in a consistent proportion of adult patients which would be considered refractory to a two-month course with corticosteroids.

Sixteen adult patients with Schönlein-Henoch nephritis, selected by strict inclusion criteria, were studied retrospectively. At the time of discovery of the nephropathy, 11 patients had normal plasma creatinine and 5 other patients had renal insufficiency. All patients had renal biopsies, which were studied by both light and immunofluorescence microscopy. After a mean follow-up of 90.5 +/- 59.1 months (range 16-261), 3 patients were in chronic dialysis (18.7%), 8 other patients had renal function deterioration (50%), with creatinine clearance ranging from 31 to 60 ml/min. Four other patients had mild urinary abnormalities with normal plasma creatinine (25%) and only 1 patient was in complete clinical remission (6%). No clinical features at onset were predictive for the clinical outcome of the disease, while in the biopsies the percentage of crescents was higher in patients who developed renal insufficiency. High IgA serum levels were correlated (p = 0.0242) with a favorable course. It is concluded that Schönlein-Henoch nephritis of the adult carries a high long-term risk of renal dysfunction.