Fibromuscular dysplasia
|
Fibromuscular dysplasia Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
ASA/ACCF/AHA Guideline Recommendations |
|
Management of Patients With Fibromuscular Dysplasia of the Extracranial Carotid Arteries |
|
Case Studies |
|
Fibromuscular dysplasia On the Web |
|
American Roentgen Ray Society Images of Fibromuscular dysplasia |
|
Risk calculators and risk factors for Fibromuscular dysplasia |
Fo patient information page, click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-in-Chief: Mohsen Basiri M.D.
Synonyms and keywords: FMD
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.
Fibromuscular dysplasia with involvement of extrarenal arteries has been considering in recent years. However numerous aspects of molecular biology ang genetic etiology of this condition remains unanswered, and there are various top research priorities in the field of FMD to improve our understanding of this condition.
Pathophysiology
- In Fibromuscular dysplasia, the proliferation of vascular smooth muscle of one or more small or medium-sized arteries undergo 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.
Causes
The cause of fibromuscular dysplasia has not been identified, nevertheless, the disease is more common among the first-degree relatives of patients with FMD, Genetic factors may play a role in the development of FMD.
Differentiating Fibromuscular dysplasia from other Diseases
Epidemiology and Demographics
- The prevalence of FMD in the general population is not known; however, the prevalence of renal artery FMD has been estimated to be as high as 4 per 100 adults.
- Patients of all age groups may develop FMD, but women are more commonly affected by FMD than men. The women to men ratio is approximately 9:1.5.
Risk Factors
There are no established risk factors for fibromuscular dysplasia; neverteless there are evidences that cigarette smoking, hypertension and other classic risk factors for atherosclerosis may be risk factors in the development of FMD .
However FMD has a greater prevalence among women but no definite association has been found between this condition and use of oral contraceptives or disturbances of endogenous sex hormones.
Since the disease is more common among the first-degree relatives of patients with FMD, Genetic factors may play a role in the development of FMD.dysplasia,
Natural History, Complications and Prognosis
- Current knowledge about natural history, the efficacy of medical therapies, complications and prognosis of FMD, are really restricted; however the natural history of FMD is benign.
- Renal or cervival artery dissection and Intracranial aneurysms rupture and SAH are most common complications of FMD
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Arteriography | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Management Guidelines | Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies