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| Fibromuscular Dysplasia |
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WHAT IS FIBROMUSCULAR DYSPLASIA?
Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease in which at least one of your arteries has an abnormal cluster of cells growing in the artery wall. This cluster causes the artery to narrow (stenosis), such as arteries in the kidneys (renal artery), arteries to the brain (carotid artery/vertebral artery) and less commonly the arteries that supplies the abdomen (abdominal aorta), which can cause damage to the organs that, receive blood through the narrowed artery. FMD is so poorly understood by many healthcare providers, treatment is often not optimal, leading to impaired quality of life and potential serious number of complications, such as hypertension, stroke, myocardial infarction (heart attack), aneurysms (arterial swelling) and arterial dissection, if left untreated. This can cause symptoms if the blood flow is decreased enough. In some patients, the diagnosis of FMD can lead to invasive procedures such as percutaneous angioplasty, reconstructive surgery or intracranial aneurysm clipping. Thus, both the disease and its treatment can lead to significant morbidity. However, FMD is often diagnosed incidentally in the absence of any signs or symptoms during an imaging study. When the vessel is filled with dye for an X-ray, it will show a characteristic “string of beads” appearance.
 Fibromuscular dysplasia is a seldom seen arterial disease characterized by abnormal cellular development or growth. Fibromuscular dysplasia commonly occurs in the renal arteries, the ones that supply blood to the kidneys. About 75% of the patients diagnosed contracted the disease on these parts. The second most common organ affected by fibromuscular dysplasia is the brain. Such condition is called carotid fibromuscular dysplasia. It is most often diagnosed in women, a fact that has led researchers to theorize that there is a connection between FMD, as it is known in short, and hormones. A second postulation is that the disease is a genetically based development disorder. These theories—and others—remain exactly that, and the disease’s cause remains unknown at this time. However, there is evidence that chronobiology -based treatments may have a role in managing fibromuscular dysplasia.WHAT ARE THE SYMPTOMS OF FMD?Most people who have FMD don't have any symptoms. Still, it's possible you could have some signs or symptoms of the disease, depending on what artery is affected by Fibromuscular Dysplasia.
Kidney Signs and Symptoms
If the arteries to your kidneys (renal arteries) are affected, you may have:
Brain Signs and Symptoms
If the arteries to your brain (carotid arteries) are affected, you may have:
Abdominal Signs and Symptoms
If the arteries to your abdomen (mesenteric arteries) are affected, you may have:
Arm and Leg Signs and Symptoms
If the arteries to your arms or legs (peripheral arteries) are affected, you may have:
Some people with fibromuscular dysplasia have more than one narrowed artery. If you have any of the signs or symptoms listed and are concerned about your risk of FMD, see your doctor. Because fibromuscular dysplasia tends to be hereditary, tell your doctor about your family history of the disease, even before you show any symptoms so that he or she can be alert to changes that might suggest you have fibromuscular dysplasia. There's currently no genetic test for fibromuscular dysplasia. And, screening for fibromuscular dysplasia isn't possible either, since it can occur in any of your arteries.
WHAT CAUSES FMD?While the cause of fibromuscular dysplasia is unknown, it's believed that several factors may play a role.
WHAT ARE THE RISK FACTORS OF FMD?Although the cause of FMD is unknown, there are several factors that appear to increase the risk of developing the disorder, including:
WHAT ARE THE COMPLICATIONS OF FMD?Fibromuscular dysplasia can cause a number of complications. These include:
HOW IS FMD DIAGNOSED?Unlike more common diseases such as diabetes or cancer, FMD is not clearly symptomatic. Diagnosis is often stumbled into after arterial condition has deteriorated so as to manifest a vascular event such as high blood pressure, kidney failure, or stroke.
The tests you'll have to diagnose fibromuscular dysplasia could include:
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 a Doppler ultrasound exam or a CT angiogram every six to 12 months to see if your condition is getting worse.
50% of Patients with Extra-Cranial Cerebrovascular FMD havean associated Intra-Cranial Aneurysm
HOW IS FMD TREATED?Treatment for fibromuscular dysplasia depends on your health, the location of the narrowed artery and other underlying conditions you have, such as high blood pressure. Treatment options include medical procedures, surgery and medications.
Medical Procedures and Surgery
For otherwise healthy people with fibromuscular dysplasia, repairing the affected artery is often recommended. The procedures to improve blood flow can include:
If your doctor finds serious damage related to fibromuscular dysplasia, such as an aneurysm, he or she may recommend placing a metal mesh tube (stent) inside the weakened part of the artery to help prevent it from rupturing.
Drugs
Treatment with high blood pressure medications is recommended for most people with fibromuscular dysplasia, even if you also have a procedure to correct your condition. These could include medications from several categories:
Because some of these drugs can affect the way your kidneys work, your doctor may recommend blood tests and a urine test (urinalysis) to make sure your kidneys work normally. If you're a smoker, another part of your treatment plan is to stop smoking. Smoking makes fibromuscular dysplasia worse.
There is no standard protocol to treat FMD. Any treatment to improve blood flow is based on the arteries affected and the progression and severity of the disease. The carotid arteries should be tested if FMD is found elsewhere in the body since carotid involvement is linked to an increased risk of stroke. Patients with minimal narrowing may take a daily antiplatelet such as an aspirin or an anticoagulant to thin the blood and reduce the chances that a clot might form. Medications such as aspirin can also be taken for headache and neck pain, symptoms that can come from FMD. Patients with arterial disease who smoke should be encouraged to quit as smoking worsens the disease. Further treatment may include angioplasty, in which a small balloon is inserted through a catheter and inflated to open the artery. Small tubes called stents may be inserted to keep arteries open. Surgery may be needed to treat aneurysms that have the potential to rupture and cause bleeding within the brain.
The only form of management for FMD is repeated screenings to monitor patients’ arteries and for patients to foster good health practices common to other vascular patients such as not smoking and eating a balanced diet. However, patients and doctors would be well advised to implement precautions based on biological rhythms into routine FMD management.
HOW MANY PEOPLE ARE AFFECTED BY FMD?
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