Fibromuscular dysplasia overview
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Fibromuscular dysplasia Microchapters |
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Diagnosis |
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Treatment |
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ASA/ACCF/AHA Guideline Recommendations |
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Management of Patients With Fibromuscular Dysplasia of the Extracranial Carotid Arteries |
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Fibromuscular dysplasia overview On the Web |
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Risk calculators and risk factors for Fibromuscular dysplasia overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohsen Basiri M.D.
Overview
The definition of Fibromuscular Dysplasia(FMD) on the Medical Subject Headings is "an idiopathic, segmental, nonatheromatous disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries. There is a true proliferation of smooth muscle cells and fibrous tissue formation. however, this systemic arteriopathy is a noninflammatory process and is therefore not associated with inflammatory biomarkers.
According to the definition, FMD is a condition which can involve every vascular bed in the body, therefore it can cause very heterogeneous and extensive spectrum of clinical manifestations from asymptomatic involvement to devastating consequences and morbidity and mortality.
Unlike routine conception that FMD is a rare disease of middle-aged female, current data from the French and US registries showed that the awareness about FMD must be raised, and every health provider at any level should be familiar with suggestive symptoms and signs of FMD which is more frequent and more often systematic than previously thought.
Historical Perspective
Fibromuscular dysplasia was first discovered by Leadbetter and Burkland, in 1938 following evaluation of severe hypertension in a 5-year-old boy. The first histopathological description of fibromuscular dysplasia and pathologic classification for this condition was proposed in 1958 and 1971 by McCormack and coworkers, chronologically.
Fibromuscular dysplasia with involvement of extrarenal arteries has been considering in recent years. However numerous aspects of molecular biology and genetic etiology of this condition remain unanswered, and there are various top research priorities in the field of FMD to improve our understanding of this condition.
Classification
- The classification system for fibromuscular dysplasia (FMD) was first according to the arterial layer involved. (tunica intima, tunica media , or adventitia) . However, with use of transluminal percutaneous angioplasty (TPA) for treatment of FMD lesions and its preference rather than surgery, the obtaining of pathological specimens are restricted. Thus, today, FMD is a disease diagnosed radiographically and histopathological classification has been replaced by the arteriographic findings.
Pathophysiology
- In Fibromuscular dysplasia, the proliferation of vascular smooth muscle of one or more small or medium-sized arteriesundergo dysplasia and cause stenosis. this abnormal cellular development is characterized by fibrous thickening of the intima, media, or adventitia of the involved arteries; which ultimately lead to arterial narrowing.