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Fibromuscular dysplasia

Definition


Disease: Fibromuscular dysplasia Fibromuscular dysplasia
Category: Bones, joints, muscles diseases

Disease Definition:

FMD is the condition in which a person's at least one of the arteries has an abnormal cluster of cells growing in the wall of the artery. This cluster will cause the artery to narrow, which in turn will cause damage to the organs that receive blood through the narrowed artery. A number of complications could be caused due to fibromuscular dysplasia (FMD) in case it is left untreated, such as aneurysms and high blood pressure.

 

In the arteries leading to the kidneys is where fibromuscular dysplasia most commonly appears. However, this sort of dysplasia could also affect the arteries that lead to the arms, legs, abdomen and brain. Fibromuscular dysplasia could be treated effectively; however, it doesn't have a cure.
 

Work Group:


Symptoms, Causes

Symptoms:

Usually, people with fibromuscular dysplasia don’t experience any symptoms. However, depending on which artery is affected by fibromuscular dysplasia, they could have some symptoms of the disease.

 

In case someone's renal arteries are affected, which are the arteries that lead to the kidneys, they may experience:

 

  • Chronic kidney failure
  • High blood pressure
  • Tissue death in the kidney (ischemic renal atrophy)

 

In case someone's carotid arteries are affected, which are the arteries that lead to the brain, they may experience:

 

  • Neck pain
  • Dizziness
  • Chronic headaches
  • Ringing in the ears (tinnitus)
  • Temporary loss of vision or blurred vision

 

In case someone's mesenteric arteries are affected, which are the arteries that lead to the abdomen, they may experience unintended weight loss and abdominal pain after eating.

 

In case someone's peripheral arteries are affected, which are the arteries that lead to the arms and legs, he/she may experience:

 

  • Numbness
  • Cold limbs
  • Weakness
  • Skin changes in color or appearance
  • Discomfort when moving the arms, hands, legs or feet

 

The doctor should be consulted in case someone experiences any of these signs or symptoms and is concerned about the risk of fibromuscular dysplasia. Even before showing any symptoms, the patient should tell the doctor about their family history of the disease because fibromuscular dysplasia tends to be hereditary. Unfortunately, a genetic test isn’t available for fibromuscular dysplasia, and since it could occur in any of the arteries, screening isn’t possible either.
 

Causes:

It is believed that several factors might play a role in causing fibromuscular dysplasia, however, its exact cause is still not known.

Abnormally formed arteries:

Inadequate oxygen to the arteries that supply the blood vessel walls with blood could cause vessels to form abnormally, and end up causing fibromuscular dysplasia. This condition could also be caused in case the position of the arteries in the body is abnormal, or in case tobacco or a medication has caused the arteries to develop abnormally. A cluster of cells builds in the artery wall once the artery develops abnormally, narrowing it and reducing the blood flow.

 

A “string of beads” appearance of the artery is the most common form of fibromuscular dysplasia. Other forms of fibromuscular dysplasia, which are more aggressive, have a smooth appearance.

Hormones:

It is though that hormones might play a role in the development of fibromuscular dysplasia because premenopausal women have this condition more often than men do. However, this condition isn’t related to whether a woman has taken birth control pills or to how many children she has had.

Genetics:

Genetics is thought to play a role in fibromuscular dysplasia. A genetic test isn’t available yet for this disease. In case one of the family members of a person has fibromuscular dysplasia, the person may never get fibromuscular dysplasia, or they may get the condition but in a different artery, or they may have a milder case or a more severe case than their family member.
 

Complications

Complications:

A number of complications could be caused by fibromuscular dysplasia, such as:

Chronic kidney failure:

Someone may have permanent kidney injury in case they don’t get enough blood flow to their kidney because the fibromuscular dysplasia has narrowed the artery to the kidney.

High blood pressure:

High blood pressure is the most common complication of fibromuscular dysplasia. Because the narrowing of the arteries causes higher pressure on the artery walls, it could lead to heart failure or further artery damage.

Aneurysms:

The blood flow could weaken the wall of the arteries in case a person's arteries are narrowed, which could create an aneurysm. A ruptured aneurysm could be a life-threatening emergency. An aneurysm could occur with fibromuscular dysplasia in an artery that leads to the arms, legs, abdomen, kidneys or brain.

Dissected artery:

Tears in the walls of the arteries could be caused by fibromuscular dysplasia, which will cause in turn blood leakage into the artery wall.

Stroke:

A person may have a stroke in case an aneurysm ruptures in an artery to the brain, or if they have a dissected artery leading to their brain.
 

Treatments:

For most people with fibromuscular dysplasia, in addition to those people who have procedures to restore the blood flow, treatment with medications is recommended. Treatment for fibromuscular dysplasia will depend on:

 

  • The patient's health
  • The location of the narrowed artery
  • Other underlying conditions that the patient, such as high blood pressure.

 

MEDICAL PROCEDURES AND SURGERY:

Usually, people with fibromuscular dysplasia who are otherwise healthy, will be recommended repairing the affected vein or artery. Some of the procedures that improve blood flow are:

Percutaneous transluminal renal angioplasty (PTRA):

Usually, at the same time that a digital subtraction or CT angiogram is performed, so is this procedure. Once the narrowed area of the artery is shown by the dye from the angiogram, a wire is threaded to the artery and a catheter with a balloon is inserted in the narrowed area. Then, in order to open the narrowed part of the artery, the balloon is inflated. Usually, a stent is not necessary to keep the artery open, unlike the angioplasty procedures performed on people with heart disease.
In most cases, PTRA is performed while the patient is awake, however, he/she will be given a sedative to relax during the procedure. This procedure usually takes one to two hours.

Surgical revascularization:

The patient may be recommended a more invasive surgery to repair the narrowed portion of the artery in case PTRA is not an option and the narrowing of the arteries is severe. Depending on the location of the narrowed artery and how damaged the artery is, the type of surgery will be determined. The patient will be asleep during the surgery because these types of surgeries require general anesthesia.
In case other damage related to the fibromuscular dysplasia is found, such as an aneurysm, the patient may also be recommended placing a metal mesh tube (stent) inside the weakened part of the artery in order to prevent its rupture.

 

MEDICATIONS:

For most people with fibromuscular dysplasia, treatment with high blood pressure medications is recommended, even if they have a procedure to correct their condition. These could include medications from several categories:

Calcium channel blockers:

These medications prevent calcium from entering the heart and blood vessel walls; they include nifedipine or amlodipine.

ACE inhibitors:

Angiotensin-converting enzyme inhibitors stop the narrowing of the blood vessels. These medications include lisinopril, benazepril or enalapril.

Beta blockers:

These medications slow the heartbeat and block adrenaline; they include atenolol or metoprolol.

 

The patient may be recommended blood tests and a urine test (urinalysis) in order to make sure that their kidneys are still functioning well because some of these medications can affect the way the kidneys work.
 

Prognosis:

Not available

Expert's opinion

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