Fibromuscular dysplasia

Overview

Fibromuscular dysplasia is a condition that causes narrowing (stenosis) and enlargement (aneurysm) of the medium-sized arteries in your body. Narrowed arteries may reduce blood flow and affect the function of your organs.

Fibromuscular dysplasia appears most commonly in the arteries leading to the kidneys and brain. Fibromuscular dysplasia can affect other arteries, including those leading to your legs, heart, abdomen and rarely the arms.

Treatments are available, but there isn't a cure for fibromuscular dysplasia.

Fibromuscular dysplasia

In fibromuscular dysplasia, the muscle and fibrous tissues in your arteries thicken, causing the arteries to narrow. This may reduce blood flow to your organs, leading to organ damage. Fibromuscular dysplasia of the artery to the kidney (renal artery) is shown here, with a "string of beads" appearance.

Symptoms

Signs or symptoms of the disease depend on what artery is affected by fibromuscular dysplasia. Sometimes, more than artery is narrowed. Many people who have fibromuscular dysplasia don't have any symptoms.

Kidney signs and symptoms

If the arteries leading to your kidneys (renal arteries) are affected, you may have:

  • High blood pressure
  • Tissue damage in your kidneys (ischemic renal atrophy)
  • Chronic kidney failure (rarely)

Brain signs and symptoms

If the arteries leading to your brain (carotid arteries) are affected, you may have:

  • Headache
  • Dizziness
  • Temporary loss of vision
  • Pulsating ringing in your ears (tinnitus)
  • Neck pain
  • Facial weakness or numbness
  • Weakness or numbness in the limbs
  • Difficulty speaking

Abdominal signs and symptoms

If the arteries leading to your abdomen (mesenteric arteries) are affected, you may have:

  • Abdominal pain after eating
  • Unintended weight loss

Heart signs and symptoms

Fibromuscular dysplasia often occurs with tears in the walls of your coronary arteries, also called arterial dissection or spontaneous coronary artery dissection (SCAD). But the exact connection between these two conditions isn't yet clear. If the arteries leading to your heart (coronary arteries) are affected, you may have:

  • Chest pain
  • Shortness of breath
  • Sweating
  • Nausea

Leg and arm signs and symptoms

If the arteries leading to your arms or legs (peripheral arteries) are affected, you may have:

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

When to see a doctor

If you have fibromuscular dysplasia, seek medical attention immediately if you have any of these signs and symptoms:

  • Sudden changes in your vision
  • Sudden changes in your ability to speak
  • Sudden or new weakness in your arms or legs

If you have any of the other signs or symptoms listed and are concerned about your risk of fibromuscular dysplasia, see your doctor.

Fibromuscular dysplasia can be passed down through families (inherited). Talk to your doctor about your family health history so together you can be alert to changes that might suggest you have fibromuscular dysplasia. There's currently no genetic test for fibromuscular dysplasia.

Causes

The cause of fibromuscular dysplasia is unknown. However, several factors may play a role.

  • Genetics. If someone in your family has fibromuscular dysplasia, you might get the condition, too. It might occur in a different artery or you may have a milder or more severe case. Or, you may never get fibromuscular dysplasia at all. Not everyone who has fibromuscular dysplasia has a relative with the disease.
  • Hormones. Women have fibromuscular dysplasia more often than men do, so researchers think hormones may play a role in the development of the disease. But exactly how is unclear. Fibromuscular dysplasia is not linked to a woman's use of birth control pills, number of pregnancies, or age when they gave birth.
  • Abnormally formed arteries. A lack of oxygen to the arteries or the use of certain medications or tobacco can cause the arteries to develop abnormally, leading to reduced blood flow. Sometimes, the arteries may be positioned abnormally.

Risk factors

Several things can make you more likely to get fibromuscular dysplasia.

  • Sex. Women have a much greater risk of fibromuscular dysplasia than do men.
  • Age. Fibromuscular disorder tends to be diagnosed in people in their early 50s.
  • Smoking. If you smoke, quit. People who smoke appear to have an increased risk of developing fibromuscular dysplasia. For those already diagnosed with the disease, smoking increases the risk for more serious fibromuscular dysplasia.

Complications

Fibromuscular dysplasia can cause a number of complications. These include:

  • High blood pressure. A common complication of fibromuscular dysplasia is high blood pressure. The narrowing of the arteries causes higher pressure on your artery walls, which can lead to further artery damage, heart disease or heart failure.
  • Dissected artery. Fibromuscular dysplasia and tears in the walls of your arteries often occur together. This process, called arterial dissection or spontaneous coronary artery dissection (SCAD), can limit blood flow to the organ supplied by the injured artery. This can affect any artery.
  • Aneurysms. Fibromuscular dysplasia can weaken the walls of your arteries, creating a bulge called an aneurysm. If an aneurysm ruptures, it can be a life-threatening emergency. An aneurysm can occur in any artery affected by fibromuscular dysplasia.
  • Stroke. If you have a dissected artery leading to your brain or if an aneurysm in an artery to your brain ruptures, you can have a stroke. High blood pressure can also increase your risk of a stroke.

Diagnosis

Because fibromuscular dysplasia may not cause any symptoms, it's unlikely your doctor would specifically check for this condition.

If someone in your family has or had fibromuscular dysplasia or aneurysm, your doctor may recommend checking you for fibromuscular dysplasia, too. Your doctor may check for fibromuscular dysplasia if he or she hears an abnormal sound in your upper stomach area that might be caused by an aneurysm.

Your doctor will perform a physical exam and order blood tests, including blood sugar and cholesterol levels, to check for signs of atherosclerosis, another condition that can narrow your arteries.

Tests to diagnose fibromuscular dysplasia could include:

  • Duplex ultrasound. This noninvasive imaging test can determine if an artery is narrowed. During the test, an instrument called a transducer is gently pressed to your skin. It sends sound waves into your body. They bounce off cells and body structures, showing how fast your blood flows and the size and shape of the blood vessels.
  • Catheter-based angiography. During this commonly used test for fibromuscular dysplasia, a thin tube (catheter) is inserted into one of your arteries and moved until it reaches the location your doctor wants to examine. A tiny amount of dye is injected and X-rays are used to examine the location.
  • Computerized tomography (CT) angiogram. A CT angiogram gives your doctor cross-sectional images of your body. It can detect narrowing in the arteries, aneurysms, and dissections. You lie on a narrow table, which slides through a doughnut-shaped scanner. Before the test starts, you'll receive an injection of a dye, which highlights areas of the body being examined.
  • Magnetic resonance imaging (MRI). MRI uses a magnetic field and radio waves to create images of the body. It can see if you have an aneurysm or dissection. During the test, you will lie on a narrow table that slides into a tubelike machine that is open on both ends.

The most common form of fibromuscular dysplasia looks like a "string of beads" on imaging tests. Other, more-aggressive forms of fibromuscular dysplasia have a smooth appearance.

Once you've been diagnosed with fibromuscular dysplasia, your doctor may repeat imaging tests from time to time to see if your signs and symptoms change or if your condition is getting worse.

Treatment

Treatment for fibromuscular dysplasia depends on your health, the location of the narrowed artery and other health conditions you have, such as high blood pressure. Treatment options include medications, medical procedures to improve blood flow and surgery.

Drugs

Treatment with high blood pressure medications is recommended for most people with fibromuscular dysplasia, even if they also have a procedure to correct the condition. Several different types of medications are available:

  • Angiotensin-converting enzyme (ACE) inhibitors, such as benazepril, enalapril or lisinopril, help relax your blood vessels.
  • Angiotensin II receptor blockers. These medications also help relax your blood vessels. Examples of this class of medications include candesartan, irbesartan, losartan and valsartan.
  • Diuretics. These drugs, such as hydrochlorothiazide, help remove excess fluid from your body and may be used with other blood pressure medications.
  • Calcium channel blockers, such as amlodipine, nifedipine and others, help relax your blood vessels.
  • Beta blockers, such as metoprolol, atenolol and others, slow your heartbeat and block adrenaline.

Your doctor may also suggest you take a daily aspirin to reduce your risk of stroke. But don't start taking an aspirin without talking to your doctor first.

Some of these medications used to treat fibromuscular dysplasia can affect the way your kidneys work. Your doctor may recommend blood and urine tests to make sure your kidneys are working normally once you start taking these medications.

Procedures and surgery

Percutaneous transluminal angioplasty (PTA)

This procedure is preferred over surgery and usually done at the same time as a catheter-based angiogram. During an angiogram, dye is injected through a catheter into an artery. X-rays show how the dye travels through the artery, revealing any narrowed areas. A wire is threaded to the artery and a catheter with a balloon is inserted in the narrowed area. The balloon is then inflated to open the narrowed part of the artery. If your doctor finds serious damage related to fibromuscular dysplasia, such as an aneurysm, a metal mesh tube (stent) may be placed inside the weakened part of the artery to help prevent it from rupturing.

Surgical revascularization

Surgery to repair the damaged artery is rarely recommended. It's usually only donewhen there are complications. However, if PTA is not an option and the narrowing of your arteries is severe, your doctor may recommend a more-invasive approach with surgery to repair or replace the narrowed portion of the artery. The type of surgery you'll need depends on the location of the narrowed artery and how damaged the artery is.

Preparing for an appointment

Because doctor appointments can be brief and because there's often a lot of ground to cover, it's a good idea to prepare ahead of time for your appointment. Here's some information to help you get ready for your appointment, and know what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance. To diagnose fibromuscular dysplasia, your doctor may order one or more imaging tests to see your arteries. You may need to fast for several hours before the tests, depending on where the narrowed arteries are in your body.
  • Write down any symptoms you're experiencing, including any that may seem unrelated.
  • Write down key personal information, including a family history of fibromuscular dysplasia, aneurysms, heart disease, stroke or high blood pressure, and any major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements you're taking.
  • Take a family member or friend along, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Be prepared to discuss your diet and your smoking and exercise habits. If you don't already follow a diet or exercise routine, be ready to talk to your doctor about any challenges you might face in getting started.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important, in case time runs out. For fibromuscular dysplasia, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • What kinds of tests will I need?
  • What treatments are available? What do you recommend for me?
  • What's an appropriate level of physical activity?
  • How often should I be monitored for fibromuscular dysplasia?
  • I have other health conditions. How can I best manage these conditions together?
  • Should I see a specialist?
  • Is there a generic alternative to the medicine you're prescribing?
  • Are there any brochures or other printed material that I can take home with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

  • When did you begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • Does anything seem to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do you have a family history of fibromuscular dysplasia, aneurysms, heart disease, high blood pressure or other serious illness?

What you can do in the meantime

It's never too late to make healthy lifestyle changes, such as quitting smoking, eating healthy foods and becoming more physically active.


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