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Although blockade of the renin-angiotensin system (RAS) is established as the first-line therapy, additional pharmacological interventions have been explored. To formulate evidence-based recommendations tailored to the Korean healthcare setting, the Genetic Kidney Disease Study Group of the Korean Society of Nephrology conducted a systematic review evaluating four therapeutic strategies: RAS blockade, cyclosporine, mineralocorticoid receptor antagonists (MRAs), and sodium-glucose cotransporter 2 inhibitors (SGLT2i). Methods: The quality of evidence and strength of recommendations were assessed using the Grading of Recommendations Assessment, Development and Evaluation framework. Results: The review confirmed that early initiation of RAS blockade delays progression to kidney failure and improves long-term survival, with the greatest benefit when initiated at the stage of microscopic hematuria or microalbuminuria. In contrast, evidence supporting cyclosporine , MRAs, and SGLT2i remains limited, primarily derived from small observational studies with short follow-up and heterogeneous populations. Although cyclosporine and MRAs have demonstrated short-term reductions in proteinuria, neither has shown sustained renoprotective effects, and cyclosporine is associated with a substantial risk of nephrotoxicity. Evidence regarding SGLT2i is inconsistent, with insufficient data on long-term efficacy in AS. Conclusion: Accordingly, these guidelines strongly recommend early RAS blockade as the cornerstone of AS, while no recommendations are made for routine use of cyclosporine, MRAs, or SGLT2i. Well-designed clinical trials are needed to expand therapeutic options and improve long-term outcomes in AS.<\/jats:p>","DOI":"10.23876\/j.krcp.25.452","type":"journal-article","created":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T08:05:19Z","timestamp":1781683519000},"page":"447-458","update-policy":"https:\/\/doi.org\/10.23876\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical practice recommendations for the management of Alport syndrome: a joint statement of the Korean Society of Nephrology and the Korean Society of Pediatric Nephrology"],"prefix":"10.23876","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8477-0157","authenticated-orcid":true,"given":"Ji 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