What are the current guidelines for the management and follow-up of patients diagnosed with Fibromuscular Dysplasia?

Management and follow-up of fibromuscular dysplasia (FMD) require a multidisciplinary approach focused on controlling symptoms, preventing complications, and monitoring disease progression. Initial management includes blood pressure control, typically with antihypertensive agents such as ACE inhibitors or calcium channel blockers, to reduce the risk of vascular events and renal impairment NICE CG147. Antiplatelet therapy, usually low-dose aspirin, is recommended to decrease the risk of thrombotic complications, especially in patients with cerebrovascular involvement or arterial dissections NICE CG147.

Imaging surveillance is essential for follow-up, with non-invasive modalities like duplex ultrasound, CT angiography, or MR angiography used periodically to monitor arterial lesions and detect new aneurysms or stenoses NICE CG147. The frequency of imaging depends on the vascular beds involved and clinical stability but generally occurs annually or biennially. Invasive angiography is reserved for cases where intervention is planned or diagnosis remains uncertain NICE CG147.

Endovascular intervention, such as percutaneous transluminal angioplasty without stenting, is considered for patients with significant symptomatic stenosis causing hypertension or ischemia, particularly in renal artery FMD NICE CG147. Surgical options are less common but may be necessary in complex cases. Lifestyle modifications, including smoking cessation and cardiovascular risk factor management, are integral to overall care NICE CG147.

Recent literature emphasizes the heterogeneity of FMD and the need for individualized management plans. The European Fibromuscular Dysplasia Initiative highlights the importance of comprehensive vascular assessment beyond the initially affected artery, as multifocal disease is common Persu et al. 2016. They also advocate for patient registries and multidisciplinary teams to optimize outcomes and guide research Persu et al. 2016. This approach complements UK guidelines by reinforcing the need for ongoing surveillance and tailored therapy based on disease extent and patient risk factors.