Fibromuscular dysplasia: information & specialists

Leading Medicine Guide Editors
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Leading Medicine Guide Editors

Fibromuscular dysplasia (FMD for short) describes a disease from the group of cardiovascular diseases. It leads to a narrowing of the affected artery, medically referred to as stenosis, or more rarely to vascular occlusion. Here you will find further information as well as selected specialists and centers for fibromuscular dysplasia.

ICD codes for this diseases: I77.3

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Brief overview:

  • What is fibromuscular dysplasia? A non-inflammatory arterial migration disease in which various wall layers of arteries are thickened, reducing the diameter of the artery.
  • Symptoms: Often there are no symptoms. They also depend on where the disease occurs in the body. Pain, poor renal perfusion, chest tightness and intermittent claudication may also occur.
  • Causes: A genetic predisposition is assumed. Smoking, connective tissue diseases and hormonal influences are also risk factors.
  • Diagnosis: Imaging procedures can show thickened vessel walls, e.g. duplex sonography, CTA, MRA and digital subtraction angiography.
  • Treatment: It is not the disease that is treated, but the symptoms. It depends on the localization of the disease. Medication is often administered. Under certain circumstances, angioplasty or, in exceptional cases, surgery is recommended.
  • Prognosis: As the disease is often asymptomatic, treatment is not always necessary. Patients should stop smoking and have regular check-ups.

Article overview

Fibromuscular dysplasia is a non-inflammatory arterial migration disease. Various wall layers of the affected artery are thickened due to accelerated growth of muscles and connective tissue. This thickening of the arterial wall can occur throughout the entire body. The result is a narrowing of the affected artery, medically referred to as stenosis, or rarely a vascular occlusion.

According to ICD code I77.3, fibromuscular dysplasia is one of the other diseases of the arteries and arterioles.

Blutgefäß Wandschichten
The vessel wall consists of connective tissue and muscles. If they thicken, the vascular cross-section is reduced © Alex Mit | AdobeStock

Symptoms

The majority of patients suffering from fibromuscular dysplasia do not notice any symptoms. However, it is generally possible for symptoms to accompany vasoconstriction.

These include pain in the affected areas, such as

  • Headaches in the case of a stenosis in the head area or
  • intermittent claudication in the case of vascular stenosis of the pelvis or legs.

Chest tightness, angina pectoris, is also possible. This describes seizure-like pain in the chest area.

If fibromuscular dysplasia affects the renal arteries, the kidneys are insufficiently supplied with blood. This then leads to high blood pressure, which is controlled by the reduced blood flow to the kidneys. This is referred to as renal hypertension.

Causes & risk factors

The actual cause of fibromuscular dysplasia is still unknown. However, doctors assume that a genetic predisposition plays a role. It has been observed that fibromuscular dysplasia occasionally occurs more frequently in families.

Smoking is also a risk factor that favors the occurrence of the vascular disease. It has also been found that people who already suffer from various connective tissue diseases have an increased risk of fibromuscular dysplasia. These include the following diseases:

  • Ehlers-Danlos syndrome type 4
  • neurofibromatosis
  • hereditary nephritis (also known as Alport syndrome) and
  • cystic medianecrosis

There is an increased incidence of the disease in women between 30 and 50 years of age. Researchers therefore assume that hormonal influences also play a role in fibromuscular dysplasia.

In principle, however, the disease can occur at any age and in both sexes.

Examination & diagnosis of fibromuscular dysplasia

Fibromuscular dysplasia is generally diagnosed by means of vascular imaging.

One of these methods is duplex sonography, a special method from the field of ultrasound examination(sonography). It allows the doctor to measure and visualize the blood flow and thus detect changes. It is carried out without the use of radiation and can therefore be used on all people.

A constriction in the blood vessels can also be visualized using computed tomography angiography(CTA). This can also be used to visualize veins and arteries.

Magnetic resonance angiography (MRA ) is another imaging procedure that can be used here.

Both procedures help to detect vascular constrictions.

The diagnosis is particularly precise using digital subtraction angiography (DSA). This procedure uses a catheter. The doctor punctures the inguinal artery and pushes a wire with the catheter up to the stenosis. DSA is generally only used if the vascular constriction needs to be corrected, for example by means of balloon dilatation.

Treatment of fibromuscular dysplasia: therapies and specialists

The treatment of fibromuscular dysplasia is basically aimed at the symptoms that the patient is suffering from. It also depends on where exactly the vascular change is located. If there are no symptoms, treatment is not necessary. However, lifelong monitoring should be carried out.

Drug treatment of fibromuscular dysplasia is possible depending on the type and intensity of the symptoms. If, for example, the neck vessels are affected, there may be a risk of embolism. In this case, the attending physician will prescribe antiplatelet agents. These agents are intended to prevent platelets from clumping together. A well-known active ingredient in this group is ASA.

If symptoms occur, angioplasty is possible in selected cases. In this procedure, the doctor attempts to widen the affected vessel by means of balloon dilatation. The doctor uses a balloon catheter to dilate the artery. The insertion of a stent, i.e. an implant to keep the vessel open, is usually not necessary.

Ballondilatation mit Stent bei Arteriosklerose
A balloon dilatation can enlarge the vessel cross-section (here with arteriosclerosis and stent) © phonlamaiphoto | AdobeStock

If neither drug therapy nor balloon dilatation have the desired effect, surgery may be necessary. The affected vessel segment is removed and replaced, usually with autologous material. These operations belong in the hands of an experienced vascular surgeon.

Specialists in vascular diseases such as fibromuscular dysplasia are vascular specialists. These include vascular surgeons and angiologists.

Vascular surgery is an independent area of surgery, while angiology is a sub-area of internal medicine.

Progression and prognosis

In many cases, fibromuscular dysplasia progresses without symptoms. Regular monitoring of the condition is therefore usually sufficient to detect a change in good time.

Patients who have been diagnosed with a vascular disease such as fibromuscular dysplasia should urgently stop smoking. It is also advisable to have other risk factors checked regularly by a doctor. For example, it should be ruled out that

These may have a negative effect on the course of the disease.

If it is necessary to have further measures carried out, these usually have a positive effect on the symptoms. In this case, a consultation with an experienced vascular specialist is necessary.

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