Ten tips for the diagnosis and management of fibromuscular dysplasia

Abstract Fibromuscular dysplasia (FMD) is an underrecognized, non-atherosclerotic and non-inflammatory arteriopathy that predominantly affects women and can involve multiple vascular territories. 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—usually unexplained vascular events—and 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, the 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.