Management of steroid resistant nephrotic syndrome.

ABSTRACT

JUSTIFICATION There is a lack of evidence based guidelines for management of children with steroid resistant nephrotic syndrome (SRNS). PROCESS Experts of the Indian Society of Pediatric Nephrology were involved in a two-stage process, the Delphi method followed by a structured face to face meeting, to formulate guidelines, based on current practices and available evidence, on management of these children. Agreement of at least 80% participants formed an opinion. OBJECTIVES: To develop specific, realistic, evidence based criteria for management of children with idiopathic SRNS. RECOMMENDATIONS: The Expert Group emphasized that while all patients with SRNS should initially be referred to a pediatric nephrologist for evaluation, the subsequent care might be collaborative involving the primary pediatrician and the nephrologist. Following the diagnosis of SRNS (lack of remission despite treatment with prednisolone at 2 mg/kg/day for 4 weeks), all patients (with initial or late resistance) should undergo a renal biopsy, before instituting specific treatment. Patients with idiopathic SRNS secondary to minimal change disease or focal segmental glomerulosclerosis should receive similar therapy. Effective regimens include treatment with calcineurin inhibitors (tacrolimus, cyclosporine), intra-venous cyclophosphamide or a combination of pulse corticosteroids with oral cyclophosphamide, and tapering doses of alternate day corticosteroids. Supportive management comprises of, when indicated, therapy with angiotensin converting enzyme inhibitors and statins. It is expected that these guidelines shall enable standardization of care for patients with SRNS in the country.