From the Guidelines
Percutaneous transluminal renal angioplasty (PTRA) without stenting is the treatment of choice for fibromuscular dysplasia, as it can restore renal perfusion pressure and lower blood pressure. Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic vascular disease that affects multiple vascular beds, including the renal and carotid arteries, and is now recognized as a systemic disease 1. The condition can lead to significant morbidity and mortality if left untreated, including uncontrolled hypertension, recurrent flash pulmonary oedema, and worsening renal function ultimately leading to end-stage renal disease 1.
Key Considerations
- Patients with FMD should receive medical therapy to reduce blood pressure in the first instance 1
- PTRA without stenting is the preferred treatment for FMD, but RAS blockers can be used as an alternative, with careful monitoring of renal function over time 1
- Possible involvement of the carotid, coronary, and other major arteries should be considered, as FMD can lead to dissection if blood pressure is not controlled 1
- Regular monitoring with imaging is recommended, particularly for patients with aneurysms [no specific evidence provided, but based on general medical knowledge]
Treatment Approach
- Antiplatelet therapy, such as aspirin 81-325 mg daily, may be considered to prevent thrombotic complications [no specific evidence provided, but based on general medical knowledge]
- Blood pressure management is crucial to prevent end-organ damage 1
- Revascularization procedures like angioplasty may be necessary for significant stenosis causing end-organ damage 1
- Smoking cessation is essential to reduce the risk of cardiovascular events [no specific evidence provided, but based on general medical knowledge]
From the Research
Definition and Prevalence of Fibromuscular Dysplasia
- Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that primarily affects women from age 20 to 60, but may also occur in infants, children, men, and the elderly 2, 3.
- FMD is considered rare, but data suggest it may be more common than previously thought, potentially affecting as many as 4% of adult women 2.
- The true prevalence of FMD is unknown due to underdiagnosis 3.
Clinical Presentation of Fibromuscular Dysplasia
- FMD most commonly affects the renal and carotid arteries, but can occur in almost any artery in the body 2, 3.
- Common presentations of FMD include hypertension, transient ischemic attack, stroke, dissection, and aneurysm 2, 3, 4.
- Some patients may be asymptomatic, with FMD discovered incidentally through imaging or detection of an asymptomatic bruit 2, 3.
Diagnosis and Management of Fibromuscular Dysplasia
- FMD should be considered in the differential diagnosis of young people with cervical bruit, transient ischemic attack, stroke, or dissection, as well as individuals aged ≤ 35 years with onset hypertension 2.
- Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention 2, 3.
- Patients with aneurysms should be treated with a covered stent or open surgical repair 2, 3.
- A comprehensive, multidisciplinary care model and patient input are essential for optimal management of FMD 4, 5.
Research and Future Directions
- The International Registry for Fibromuscular Dysplasia aims to provide observational data on a large number of patients with FMD and remedy the lack of new information on the condition in recent decades 2, 3.
- Ongoing research programs and future research directions for understanding FMD are outlined in the First International Consensus on the diagnosis and management of fibromuscular dysplasia 6.