This is a sobering picture of the limits of evidence that define the “state of the art” in the clinical management of adult NS.Adult-onset NS differs from its pediatric counterpart in several important ways. In adults, relative contraindications to steroid use are more common (e.g., diabetes, osteoporosis), as is steroid intolerance, and in clinical decision-making individual patient considerations must trump broad practice guidelines (The anti-CD20 monoclonal antibody rituximab is not mentioned in the KDIGO guidelines on adult MCD, except in an appeal for further research. The estimated incidence is 8-10 cases per 1 million. Published by Elsevier Inc. All rights reserved. | Some additional points may be helpful. This study compared the effect of a 6-mo course of alternating prednisolone and cyclophosphamide with … Authors Rutger J … (5.1.5: For patients with relative contraindications or intolerance to high-dose corticosteroids (e.g., uncontrolled diabetes, psychiatric conditions, severe osteoporosis), we suggest oral cyclophosphamide or CNIs as discussed in frequently relapsing MCD. 2017 May;69(5):637-646. doi: 10.1053/j.ajkd.2016.10.032. No statement was supported by grade A evidence, and only one suggestion was graded B (moderate quality). The Lancet CYCLOPHOSPHAMIDE TREATMENT IN STEROID-SENSITIVE NEPHROTIC SYNDROME OF CHILDHOOD T.M. Copyright © 2016 National Kidney Foundation, Inc. Please enable it to take advantage of the complete set of features! One consideration is why these agents are considered second-line behind steroids. (5.3.1: Re-evalulate patients who are corticosteroid resistant for other causes of nephrotic syndrome.
Pediatr., 25 September 2015 2015 Jun;19(3):496-505. doi: 10.1007/s10157-014-1022-x. It should be recognized that the weakness of the evidence relates to the choice of replacement, not to the premise of withholding steroids in the first place – there need be very little “evidence” to justify NOT giving a medication which is poorly tolerated or contraindicated! There is no evidence-based approach for ruling out secondary FSGS. The rationale cites the lack of randomized trials, but also states that limited observational data suggests tacrolimus may be an alternative to cyclosporine. Elsevier Science (6.2.4: We suggest corticosteroids be tapered slowly over a period of 6 months after achieving complete remission. A Cochrane review of immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome concluded that combination treatment with an alkylating agent and a corticosteroid has both short- and long-term benefits, and that cyclophosphamide is a safer alkylating agent than chlorambucil. (5.2.2: We suggest CNI (cyclosporine 3–5 mg/kg/day or tacrolimus 0.05–0.1 mg/kg/day in divided doses) for 1–2 years for FR/SD MCD patients who have relapsed despite cyclophosphamide, or for people who wish to preserve their fertility. ScienceDirect ® is a registered trademark of Elsevier B.V.CYCLOPHOSPHAMIDE TREATMENT IN STEROID-SENSITIVE NEPHROTIC SYNDROME OF CHILDHOODScienceDirect ® is a registered trademark of Elsevier B.V. Universal consensus regarding the need for and the modality of therapy has not been formed because of a lack of controlled trials of sufficient size, quality, and duration. (5.4.2: We suggest that, for the initial episode of nephrotic syndrome associated with MCD, statins not be used to treat hyperlipidemia, and ACE-I or ARBs not be used in normotensive patients to lower proteinuria. As with all clinical practice guidelines, a critical appreciation of their limitations and an eye toward emerging lines of evidence are necessary to most effectively apply their lessons to individual patients.PC drafted the manuscript.
(6.3.1: We suggest that a relapse of nephrotic syndrome is treated as per the recommendations for relapsing MCD in adults (see Chapters 5.1 and 5.2). In recent years, the use of adrenocorticotropic hormone (ACTH) therapy for treatment of proteinuria due to nephrotic syndrome (NS) has been heavily explored. New research adds to, but rarely supplants, prior evidence, which is why venerable “tried and true” interventions such as corticosteroids continue to play such a prominent role in the recommendations. A small, single-center study of adults with idiopathic FSGS compared intravenous monthly cyclophosphamide to tacrolimus for 6 months, with both groups receiving steroids (For steroid-resistant patients intolerant to cyclosporine, KDIGO recommends only combined MMF and high-dose dexamethasone.
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