Consensus Guidelines on Management of Steroid-Resistant Nephrotic Syndrome.

Justification The management of steroid resistant nephrotic syndrome (SRNS) is challenging. These guidelines update existing 2009 Indian Society of Pediatric Nephrology recommendations on its management. Objectives To frame revised guidelines on diagnosis and evaluation, treatment and follow up, and supportive care of patients with the illness. Process The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by systematic review of literature, evaluation of evidence by experts and two face-to-face meetings. Recommendations Fourteen statements provide updated advice for defining steroid resistance, and underscore the importance of estimating proteinuria and baseline kidney function, and the need for kidney biopsy and genetic screening. Calcineurin inhibitors are recommended as most effective in inducing remission of proteinuria, the chief factor associated with long-term renal survival. Advice on managing allograft recurrence, congenital nephrotic syndrome, and monitoring and supportive care, including transition of care are described. This revised practice guideline is intended to improve management and patient outcomes, and provide direction for future research.