Fibromuscular dysplasia overview

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Fibromuscular dysplasia Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Fibromuscular dysplasia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiorgram

X-ray

CT

MRI

Arteriography

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Management Guidelines

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

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Future or Investigational Therapies

ASA/ACCF/AHA Guideline Recommendations

Management of Patients With Fibromuscular Dysplasia of the Extracranial Carotid Arteries

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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 intimatunica 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

Causes

The cause of fibromuscular dysplasia has not been identified. To review risk factors for the development of FMD, click here.

Differentiating Xyz from Other Diseases

  • There is a significant delay in diagnosis from the first onset of clinical symptoms/signs of 4.4 years in men and 4.1 years in women. There are several possible reasons for such a delay, including the possibility that FMD is not considered in the differential diagnosis of a patient’s symptoms because of under-recognition of this disorder, the mistaken belief that FMD is predominately a rare disease of young patients, and the fact that many of the signs and symptoms of FMD are nonspecific, such as dizziness, tinnitus, and headaches.

Epidemiology and Demographics

Risk Factors

There are no established risk factors for fibromuscular dysplasia; neverteless there are evidences that cigarette smokinghypertension 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.

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

Catheter-based angiography is the gold standard test for the diagnosis of renovascular fibromuscular dysplasia. Imaging modilities are the methods for diagnosing FMD. Duplex ultrasonography, accompanied by computed tomographic angiography(CTA), and magnetic resonance angiography (MRA), are imaging techniques for detecting FMD lesions but the gold standard remains catheter-based angiography.

History and Symptoms

Since Fibromuscular dysplasia can involve virtually every artery of the body, thus the clinical presentations of FMD vary widely and are determined by the vessels territories that are involved.The clinical presentations of FMD may result from the stenosis, occlusion, ischemiadissectionrupture of aneurysms and embolization of intravascular thrombi from dissection or aneurysms.

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Revascularization by surgical intervention or percutaneous transluminal angioplasty (PTA) , are not the first-line treatment options for patients with FMD, however in cases of FMD-related RAS with signs of downstream reduction of renal function or in case of drug-resistant HTN vascular reconstruction should be considered.

Primary Prevention

There are no established measures for the primary prevention of fibromuscular dysplasia.

Secondary Prevention

References


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