TREATMENT OF SEVERE NEPHROTIC SYNDROME

Treatment modalities in severe nephrotic syndrome have to consider (a) the underlying glomerular diseases as well as (b) the extrarenal comp lications. Occasionally acute renal failure develops on the basis of a n unknown nephrotic syndrome; if a primary glomerular disease is diagn osed by biopsy, immunosuppressive therapy is optional. In type I and t ype II diabetes development of a severe nephrotic syndrome is usually not reversible. To avoid the rapid decline of renal function a consequ ent antihypertensive therapy is the treatment of choice in this stage of the disease. Treatment of primary glomerular diseases with severe ( NS) includes frequently relapsing minimal change nephropathy (MCN) tha t can be treated with prednisolone 1 mg/kg/day until remission occurs. For prolongation of the remission cyclophosphamide 2 mg/kg/day for ei ght weeks, or alternatively cyclosporine A 3 to 5 mg/kg/day for six mo nths, can be given. In steroid-resistant focal segmental glomeruloscle rosis (FSGS) eight weeks of treatment with cyclophosphamide 2.5 mg/kg/ day or six months treatment with cyclosporine A 3 to 5 mg/kg/day can i nduce a partical or complete remission in up to 20% of the patients. I n membranous glomerulopathy with severe NS, one month of therapy with prednisolone followed by chlorambucil for one month (all together 6 mo nths) improves the renal outcome of the patients compared to controls. Alternatively, cyclophosphamide 2 mg/kg/day plus 30 mg prednisolone/d ay can be given for a couple of months. Extrarenal complications of a severe NS are: (a) edema; (b) thromboembolism; and (c) lipid abnormali ties. If nephrotic patients are resistant to orally administered loop diuretics, they should be treated in addition intravenously with hydro chlorothiazide p.o. Nephrotic patients with a serum albumin level < 20 g/liter should be routinely anticoagulated. Extensive hyperlipidemia in severe NS can be treated with HMG-CoA reductase inhibitors.