A vascular disease called fibromuscular dysplasia, which can cause high blood pressure, kidney failure, stroke and other symptoms -- mostly in women -- is "poorly understood by many healthcare providers," according to a Scientific Statement from the American Heart Association.
Neurologist Jose Biller, MD, of Loyola University Medical Center, is a co-author of the statement, published online ahead of print in the American Heart Association journal Circulation. First author is Jeffrey W. Olin, DO of Mount Sinai School of Medicine.
Biller said FMD "is a relatively uncommon and often undiagnosed arterial condition of unknown cause."
Biller is an internationally known stroke specialist and chair of the Department of Neurology of Loyola University Chicago Stritch School of Medicine. He is a member of the multidisciplinary group of vascular experts who wrote the American Heart Association's Scientific Statement on fibromuscular dysplasia (FMD).
FMD can cause narrowing, enlargement, bulging or tears in medium-size arteries. FMD occurs most commonly in arteries leading to the kidneys, and in carotid arteries in the neck that carry blood to the brain and eyes. It also can affect arteries supplying blood to the abdominal organs, legs or arms. Neurological complications include headaches, neck pain, tinnitus (swishing sounds in the ears), strokes and transient ischemic attacks (mini strokes). FMD may be a predisposing condition for cervical arterial dissections (tears in neck arteries). It also can cause brain aneurysms or bleeding on the surface of the brain.
FMD "is poorly understood by many healthcare providers," the Scientific Statement said. A delay in diagnosis "can lead to impaired quality of life and poor outcomes."
The condition was first described in a medical journal in 1938, and given the name fibromuscular dysplasia in 1958.
Although the prevalence in the general population is unknown, 91 percent of patients with FMD are female, the Scientific Statement said. Although the cause is unknown, there appears to be a genetic basis for susceptibility to FMD.
The Scientific Statement lists several common misconceptions regarding FMD. One misconception is that the most common presentation for FMD in the carotid artery is a stroke or mini stroke. In fact, while strokes and mini strokes can occur with carotid FMD, the most common presentations are nonspecific symptoms such as headaches, dizziness, light-headedness and tinnitus.
FMD often is misdiagnosed as other conditions, such as atherosclerosis and vasculitis. The gold standard for correctly diagnosing FMD is a catheter-based angiogram.
Advances in imaging and therapies "have made the treatment for patients with FMD less invasive, safer and more effective," the Scientific Statement said.
But there is a great need for more research, the statement said. "Significant advances in our understanding of FMD will undoubtedly require collaboration across a large network of research and clinical centers in the United States and abroad," the statement said.
The Fibromuscular Dysplasia Society of America praised the Scientific Statement. "The authors did a great job and covered all areas of FMD, including history of the disease, diagnosis, imaging, treatment and research," the society said on its website.
- J. W. Olin, H. L. Gornik, J. M. Bacharach, J. Biller, L. J. Fine, B. H. Gray, W. A. Gray, R. Gupta, N. M. Hamburg, B. T. Katzen, R. A. Lookstein, A. B. Lumsden, J. W. Newburger, T. Rundek, C. J. Sperati, J. C. Stanley. Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions: A Scientific Statement From the American Heart Association. Circulation, 2014; DOI: 10.1161/01.cir.0000442577.96802.8c
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