What is the typical patient profile?

The typical patient profile is ……. a woman aged between 20 and 40 years, diagnosed with hypertension, in the absence of being overweight or a family history of hypertension. Women with FMD often complain of headaches/migraine and pulsatile tinnitus (a whooshing sound) which could signal a cerebrovascular (head/neck arteries) form of FMD. It is worth remembering that these symptoms can have many causes other than FMD, which is still considered a relatively rare disease. FMD in its less severe form may be discovered incidentally, when imaging is being carried out for an unrelated reason. The average age at diagnosis of FMD is around 50 yrs. Men, children and the elderly may also be diagnosed with FMD.

If I have FMD must I limit my fitness/sporting activity?

Currently there are no studies to suggest patients with FMD should limit their fitness or sporting activity. Looking at the FMD patient studies, there have been very few incidents where a sporting activity can be associated with a complication of the disease. That said, arterial dissections, most notably carotid or coronary artery dissections, whether FMD-related or not can be seen after intense sporting or physical activity, or abrupt movement. Mechanical factors may also play a role in aneurysm rupture. It is therefore recommended that patients should follow an active lifestyle with regular exercise, but for those with aneurysm or a history of dissection avoiding intensive, contact or resistance sports would be advisable.

Should I stop smoking?

Tobacco is a major risk factor of heart disease and death. This argument alone is sufficient to recommend smoking cessation. Furthermore, for patients with FMD, studies show worse outcomes for smokers. It is thought that patients with FMD who smoke may suffer more severe arterial lesions. It is also felt that smoking may increase the risk of progression in existing FMD. It is therefore strongly recommended that patients with FMD give up smoking.

What is the advice concerning oral contraception or HRT (menopause treatment)?

A role of female hormones in FMD may be suggested by the higher number of women affected.
However, to date there is no firm evidence that contraception, HRT (Hormone Replacement Therapy) or pregnancy increase the development or the progression of FMD.

So, in the absence of demonstration that oral contraceptives or HRT have aggravating effects on FMD, these medications are not formally contraindicated in women with FMD.

Can renal artery FMD increase the risk of complications during pregnancy?

FMD of the renal arteries often affects younger women of childbearing age. A stenosis (narrowing) of the renal arteries may be the cause of hypertension. Hypertension may increase during pregnancy, it could also possibly increase the risk of pre-eclampsia or eclampsia which could pose a risk to the mother and her child.
Renal artery FMD can also be associated with FMD lesions in other affected arteries such as the carotid and intracranial arteries. It may be there is an increased risk of dissection or arterial bleeding during pregnancy.

However, these types of complications remain rare and the vast majority of patients with FMD will have a pregnancy and delivery without FMD-related complications.

Recommendations for patients with FMD considering pregnancy:

• Have your blood pressure checked regularly by a specialist with experience of hypertension in pregnancy
• If not already done, consult a specialist to screen for aneurysms or dissections before pregnancy in order to avoid possible complications during the pregnancy and delivery.
• Avoid intensive exercise during your pregnancy. However, regular gentle physical exercise
(walking, cycling or swimming) is recommended and beneficial.

Does FMD progress or worsen over time?

In the majority of cases, FMD is stable, and if it does progress, it does so slowly.
In a minority of cases – most notably, but not exclusively in the case of FMD in children and young adults, there can be a rapid progression of the FMD lesions, with sometimes dramatic complications. Bilateral renal artery (affecting both renal arteries) or focal FMD (one or several isolated stenosis, not the string of beads type) seem more likely to progress.

The multicentre French study PROFILE initiated by Prof. PF Plouin (HEGP, Paris) – to which Prof. A Persu and Prof. P Van der Niepen participated , will allow the proportion and characteristics of patients who experienced progression of existing FMD lesions, or developed new FMD lesions over a three year period to be seen.
Registries such as the European FMD registry will allow an expanding and refining of these findings in larger numbers of patients followed for longer periods of time.

An annual follow-up is recommended for all patients diagnosed with FMD (see next question)

What follow-up is necessary for FMD patients?

FMD is a chronic vascular disease. Therefore, a yearly follow-up appointment is recommended, preferably in a centre experienced in following patients with FMD.
Follow-up should include a clinical examination with blood pressure measurement, blood test to check renal function (in the case of renal FMD) and medical imaging. The frequency and exact nature of these tests is determined on a case-by-case basis, according to the vascular beds involved and the severity of the involvement.