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It commonly presents with hypertension, cerebrovascular events, or arterial dissections in otherwise low-risk individuals, yet diagnostic delays and management variability remain frequent. This ten tips review provides practical guidance for clinicians to improve recognition, diagnosis, and management of FMD in everyday practice. Key recommendations emphasize maintaining a high index of suspicion in patients with early-onset or resistant hypertension, pre-eclampsia, pulsatile tinnitus, or unexplained headaches. High-quality imaging with computed tomographic angiography or magnetic resonance angiography is central to diagnosis, alongside a one-time head-to-pelvis vascular assessment to detect multivessel involvement. The review highlights the importance of recognizing atypical presentations\u2014usually unexplained vascular events\u2014and the limited role of routine genetic screening, while advocating selective evaluation of at-risk relatives. Management strategies, including percutaneous transluminal angioplasty, focus on individualized care. Medical management prioritizes strict blood pressure control, smoking cessation, and selective use of antiplatelet therapy, while statins are not routinely indicated. Lifestyle guidance supports regular physical activity with avoidance of high-risk activities that may predispose to dissection. Special considerations include pregnancy, which is generally safe with appropriate monitoring, and migraine management, which requires balancing efficacy with vascular safety. Finally, this review underscores the importance of multidisciplinary care, patient education, and participation in registries and support networks to advance knowledge and improve outcomes in this rare disease.<\/jats:p>","DOI":"10.1093\/ckj\/sfag234","type":"journal-article","created":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T11:24:06Z","timestamp":1783941846000},"source":"Crossref","is-referenced-by-count":0,"title":["Ten tips for the diagnosis and management of fibromuscular dysplasia"],"prefix":"10.1093","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-9237-1427","authenticated-orcid":false,"given":"Julie","family":"Piotte","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine, Centre Hospitalier Universitaire de Sherbrooke (CHUS), Universit\u00e9 de Sherbrooke , Sherbrooke, Qu\u00e9bec ,","place":["Canada"]},{"name":"Division of Cardiology, Department of Cardiovascular Diseases, Cliniques Universitaires Saint-Luc and Pole of Cardiovascular Research, Institut de Recherche Exp\u00e9rimentale et Clinique, Universit\u00e9 Catholique de Louvain , Brussels ,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Simona","family":"Stoenoiu","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Cliniques Universitaires Saint-Luc, Universit\u00e9 Catholique de Louvain , Brussels ,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cathlin","family":"Jamison","sequence":"additional","affiliation":[{"name":"European Fibromuscular Dysplasia Patient Association (FMD-EU) , Brussels ,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3825-6955","authenticated-orcid":false,"given":"Alexander","family":"Woywodt","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine, Lancashire Teaching 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