Main clinical recommendations in 2019
Here are the main recommendations from the expert panel at the origine of the “First International Consensus on the Diagnosis and Management of Fibromuscular Dysplasia”:
1. FMD lesions should be classified according to angiographic appearance as multifocal (string of beads appearance) or focal (one or several isolated stenosis).
2. There are currently no genetic tests that are specific to FMD, and there is no justification for genetic testing of patients with FMD at this time. Pending future genetic developments, relatives of patients with FMD should only undergo clinical examination and image-based evaluation of potentially affected arterial beds upon presentation with suggestive symptoms or signs of FMD.
3. The diagnosis of FMD requires medical imaging.
If renal artery FMD is suspected, the first-line recommended imaging is CTA, or if contraindicated MRA. Duplex ultrasound as a diagnostic tool is best reserved for FMD experienced centres.
CTA or MRA are also the preferred tests in the diagnosis of cerebrovascular FMD
(carotid/vertebral). It is worth noting that duplex ultrasound does not allow visualizing the distal portion of carotid arteries (a frequent location of carotid FMD), or the vertebral or intracranial arteries.
4. Regardless of the initial site(s) of the FMD lesions, all patients with FMD should undergo a one-time assessment for intracranial aneurysms with either a brain CTA or MRA.
5. Regardless of the initial site of FMD lesions, all patients with FMD should undergo a one-time imaging of all vessels from brain to pelvis, usually with CTA or contrast-enhanced MRA to identify other areas of FMD, as well as to screen for aneurysms and dissections.
6. In the absence of contraindication, antiplatelet therapy (i.e. aspirin 75-100 mg daily) is reasonable for patients with FMD in order to prevent thrombotic or thromboembolic complications (complications from blood clots). Prescription of aspirin should nevertheless be weighed according to each patient’s medical history and risk profile.
7. All patients who suffered a SCAD should undergo a one-time imaging of all vessels from brain to pelvis, usually with CTA or contrast-enhanced MRA, to identify FMD lesions and other arterial abnormalities.
8. Patients with FMD should be followed-up at least annually, preferably in a specialized centre. Follow-up includes clinical assessment, evaluation of renal function (blood test, in the case of renal FMD) and imaging. The timing and nature of imaging should be determined on a case-by-case basis according to the severity of the disease.