Fibromuscular dysplasia pathophysiology
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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 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 true proliferation of SMOOTH MUSCLE CELLS and fibrous tissue. however this type of angiopathy is a noninflammatory process and is therefore not associated with inflammatory biomarkers.
according the definition FMD is a condition which can involve every vascular bed in the body, therefor 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 disease of middle-age female current data from the French and US registries showed that irrespective of age and sex, every practitioner at any level should be familiar with suggestive symptoms and sings of FMD and raise awareness about FMD which is more frequent and more often systematic than previously thought.
Pathophysiology
In individuals with FMD, the walls of one or more arteries undergo dysplasia. Due to this abnormal cellular development, the vessels may become stenosed. A sufficient decrease in blood flow through the artery can cause symptoms. Fibromuscular dysplasia is characterized by fibrous thickening of the intima, media, or adventitia of the renal artery. Up to 75% of all patients with FMD will have disease in the renal arteries. The lesions cause narrowing of the artery lumen. The second most common artery affected is the carotid artery, which is found in the neck and supplies the brain with blood. Less commonly, FMD affects the arteries in the abdomen (supplying the liver, spleen and intestines) and extremities (legs and arms). More than one artery may have evidence of FMD in 28% of people with this disease. All arteries should be checked if FMD is found in one vascular bed. As a result of renal artery stenosis, the kidney's afferent arteriolar pressure falls. The renin-angiotensin system is activated, causing fluid retention and hypertension.
Pathogenesis
- The exact pathogenesis of [disease name] is not fully understood.
OR
- It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
Genetics
- [Disease name] is transmitted in [mode of genetic transmission] pattern.
- Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
- The development of [disease name] is the result of multiple genetic mutations.
Associated Conditions
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].