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What is FMD?
Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic arterial disease that is most commonly seen in women. It may present at any age, but is more commonly discovered when the patient is between the ages of 20 and 60 years old. Patients may be asymptomatic and remain undiagnosed; however, the disease may cause arterial stenosis, occlusion, aneurysm, and/or dissection. FMD most commonly impacts the renal and carotid arteries, though it has been identified in almost every arterial bed.

FMD is considered a rare disease. In the U.S., a disease is defined as rare if it is believed to affect fewer than 200,000 Americans, according to the National Organization of Rare Disorders. A disease or disorder is defined as rare in Europe when it affects less than 1 in 2,000. The disease has no cure.

What causes FMD?
It is unknown what causes FMD. Researchers believe that the disease may have hormonal and genetic influences.

When was FMD first discovered?
"Fibromuscular dysplasia (FMD) was first described in 1938 in the renal arteries as a cause of arterial hypertension in a patient with unilateral kidney disease who had “an intraluminal mass of smooth muscle” (Leadbetter and Burkland, 1938; Sandok, 1989; Slovut and Olin, 2004). More than two decades later, McCormack et al. (1958, 1966) described the renal artery pathology of FMD. For many years, fibromuscular disease was assumed to be limited to the renal arteries. Palubinskas and Ripley (1964) were the first to report the angiographic appearance of FMD in extrarenal arteries. Their patient had involvement of the celiac artery and an angiographic lesion in an internal carotid artery (ICA). A year later, Javid (1965) first furnished histological proof of FMD in a carotid artery (Sandok, 1983). Connett and Lansche (1965) were the first to describe the radiological appearance of carotid artery FMD in a patient in whom the disease was confirmed pathologically," according to Uncommon Causes of Stroke, Cambridge University Press.

How common is FMD?
FMD is considered a rare disease. In the U.S., a disease is defined as rare if it is believed to affect fewer than 200,000 Americans, according to the National Organization of Rare Disorders. A disease or disorder is defined as rare in Europe when it affects less than 1 in 2,000. Research extrapolates that the disease may be found in as much as 5 percent of the U.S. population of women over 18—or 5 to 8 million women; however, at this time these figures are indicated as estimates only, as reported in the February 2012 Endovascular Today article “Renal Artery Fibromuscular Update.”

What type of FMD is most common?
Most cases of FMD are of the medial fibroplasia type, which commonly is described as giving the affected artery a string of beads appearance upon angiogram. The next most common type of FMD is intimal fibroplasia. Adventitial fibroplasia is uncommon.

What is the most common clinical presentation of FMD?
Hypertension is the most common clinical presentation of FMD when patients’ renal arteries are affected. This hypertension often presents without other obvious causes and is resistant to control via medication. Note that diagnosis of FMD may occur at any age. As reported by researchers at Universidade Federal da Paraíba in the article “Fibromuscular dysplasia: a differential diagnosis of vasculitis,” published in Rev Bras Reumatol, “FMD can manifest as a systemic vascular disease, mimicking vasculitis. This understanding is important because vasculitis and FMD can both have a severe clinical course, but require distinct treatments. The differential diagnosis can be difficult in face of an atypical clinical presentation or lack of histopathologic confirmation. Isolated cases of FMD have been reported mimicking the following conditions: polyarteritis nodosa, Ehlers-Danlos's syndrome, Alport's syndrome, pheochromocytoma, Marfan's syndrome, and Takayasu's arteritis.”

How is FMD evaluated?
Catheter-based angiography is the most accurate imaging method. Other less invasive imagining methods such as duplex ultrasound, magnetic resonance angiography (MRA) and computed tomography angiography (CTA) also may be considered. Doctors are advised not to rely only on imaging for diagnosis, as presentations may be subtle. According to research published in Endovascular Today, “Although there have been significant strides made during the past several years toward educating physicians and patients about the disease, FMD still remains frequently underdiagnosed, misdiagnosed, and inappropriately treated” (February 2012).

What treatment is available for FMD?
Angioplasty, stenting, and reconstructive vascular surgery all are used in managing the effects of the disease. Patients also may be prescribed blood pressure and/or antiplatelet medications. “Most patients today acquire the diagnosis via an imaging study and can be treated with medication. This strategy can minimize symptoms but does not ‘cure’ the disease,” says Dr. Bruce H. Gray, D.O., of the Greenville Hospital System University Medical Center and Chief Operating Officer of the American Board of Vascular Medicine.

FMD by the numbers*

Migraine-like Headache—53 percent of patients
Dizziness—28 percent of patients
Tinnitus—30 percent of patients
Extracranial Carotid Involvement—62 percent of patients
Vertebral Artery Assessment—19 percent of patients
Carotid Dissection—14 percent of patients
Carotid Aneurysm—4 percent of patients
Mesenteric Artery Involvement—12 percent of patients
Renal Artery Involvement— 69 percent of patients
Renal Artery Aneurysm—5 percent of patients
* Percentages as reported by Dr. Jeffery W. Olin at the 2012 International Symposium on Endovascular Therapy based on baseline observations from the U.S. Registry for Fibromusuclar Dysplasia; study group comprised of 339 patients.

Fibromuscular Dysplasia
Cleveland Clinic

Renal Artery Fibromuscular Dysplasia Update, Dr. Joe F. Lau et al
Endovascular Today (2012)

Fibromuscular Dysplasia, Plouin et al
Orphanet Journal of Rare Disease (2007)

Fibromuscular Dysplasia: a differential diagnosis of vasculitis, Universidade Federal da Paraíba
U.S. National Library of Medicine (2012)

Fibromuscular Dysplasa, Dr. Ido Weinberg
Vascular Medicine (2010)

Fibromuscular Dysplasia Imaging
Medscape Reference (2011)

Spontaneous Coronary Artery Dissection in Patients with Fibromuscular Dysplasia, Jacqueline Saw, M.D. et al
American Heart Association (2012)

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THE BODY AND DISEASE COMPLICATIONS

Understanding the kidneys and how they work
Understanding the cardiovascular system
High blood pressure dangers: Hypertension's effects on your body
Understanding stroke and transient ischemic attacks
What is an aneurysm?
What is an arterial dissection?
About dizziness
About Pain

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FMD CHAT'S NEEDYMEDS PRESCRIPTION DRUG DISCOUNT CARD



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The drug discount card is not an insurance plan, but provides patients with discounted rates on prescriptions. When this card is presented with a valid prescription to a pharmacist, up to an 80 percent discount may be provided. For more information about NeedyMeds, visit needymeds.org. Frequently asked discount card questions and their answers can be found here.
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 MEDICAL IDENTIFICATION TAGS
Shop at Sticky J Jewelry and 10 percent of your order's total will go FMD Chat—note that only purchases made from FMD Chat's specific link count. Visit Sticky J for Medical ID Alert Jewelry.
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UNDERSTANDING ONLINE HEALTH

Evaluating Internet Health Information: A Tutorial
U.S. National Library of Medicine and National Institutes of Health

MedlinePlus Guide to Healthy Web Surfing
U.S. National Library of Medicine and National Institutes of Health

Medical Dictionary
U.S. National Library of Medicine and National Institutes of Health
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CAREGIVING

Taking Care of YOU: Self-Care for Family Caregivers
Family Caregiver Alliance

MedlinePlus Caregiving
U.S. National Library of Medicine and National Institutes of Health

National Caregiving Library
FamilyCare America, Inc.

Palliative Care vs. Hospice
National Hospice and Palliative Care Organization
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ADVANCE PLANNING

End-of-Life Decision Making
Family Caregiver Alliance

National Healthcare Decisions Day

Advance Directives
National Hospice and Palliative Care Organization
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INTERNATIONAL RARE DISEASE ORGANIZATIONS


Australia
SMILE

Austria
Pro Rare Austria

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