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What Is Fibromuscular Dysplasia? Being a poorly understood cardiovascular disease, Fibromuscular dysplasia (FMD) can strike women up to 10 times more than men. It can lead to many complications including hypertension, chronic kidney failure, aneurysms, and spontaneous coronary artery dissection (SCAD). It can even trigger life-threatening stroke and heart attack. SCAD is a rare kind of heart disease that occurs when a tear forms in one of the blood vessels in the heart. It can slow or block blood flow to the heart, causing a heart attack, abnormalities in heart rhythm and sudden death. Until a decade ago, FMD was a very rare disease. It was first described in a European medical journal in 1938, and research was subsequently conducted in the United States in the 1950s and 1960s. In 2009, the FMD Society of America funded a registry based at the University of Michigan, which now tracks 1,200 patients at 14 medical centers. The exact statistics on how many Americans have FMD is unavailable. FMD is a non-atherosclerotic, non-inflammatory disease of the blood vessels. Patients with FDM have abnormal growth within the wall of an artery that can cause narrowing, aneurysms or tears in the arteries. Arteries affected by FMD have a classic “string of pearls” appearance. It can be found in nearly every arterial bed in the body but the most common arteries affected are the renal and carotid arteries. The renal arteries are the blood vessels that carry blood from the aorta to the kidneys, and the carotid arteries are in the neck and connect the heart and the brain. About 65 percent of the time, it affects the renal arteries causing uncontrolled hypertension. Approximately 10 percent of the cases appear to be inherited and often coexist with other genetic abnormalities that affect the blood vessels, according to the Cleveland Clinic. Nevertheless, cause of FMD is believed not to be a single identifier but has multiple underlying factors. For instance, it could be caused by hormonal influence, mechanical stress from trauma and stress to the artery walls, and it could also be the effect of loss of oxygen supply to the blood vessel wall caused by fibrous lesions. It has been suggested that environmental factors like smoking and estrogen might play a role, too.
Patients with FMD may have symptoms like headaches, especially migraine type headaches, a pulsatile swooshing noise in the ears, neck pain and lightheadedness. They may also be entirely asymptomatic. Women aged between 30 and 50 are at greatest risk for FMD though it could also affect children and the elderly. In a study from the US Fibromuscular Dysplasia registry, the mean age at first symptom or sign was about 47 years of age. The average span from the first symptom to FMD diagnosis, as revealed by a 2014 study in ‘Circulation’ (a journal of the American Heart Association), is 4 to 9 years. Such delay could just waste precious time for proper treatment. Both invasive tests such as angiography and non-invasive tests like duplex ultrasonography, magnetic resonance angiography (MRA), and computed tomographic angiography tests (CTA) can be used to diagnose FMD. Sometimes, patients are diagnosed with FMD after having an X-ray or scan for another problem, and the doctor discovers the beaded appearance of the arteries. Some patients are diagnosed with FMD during a routine examination after the doctor hears a swooshing noise, called a bruit, which means there is abnormal blood flow. Once FMD is discovered in one part of the body, more imaging tests are usually done to check for FMD in other areas. For instance, the patient should have tests to check for an aneurysm (in the brain or aorta/aortic branches) that may require additional treatment. So far, there is no known cure for FMD. Treatment focuses on relieving symptoms associated with it. Treatment may include medications for blood pressure and clots, or vascular procedures such as angioplasty or surgery. Generally, FMD is not preventable. But if one is a smoker, quitting smoking might just reduce the likelihood of developing FMD.
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