Displasia fibromuscular: um diagnóstico diferencial para as vasculites View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2012-02

AUTHORS

Thaís de Carvalho Pontes, Geísa Pereira Rufino, Mariana Galvão Gurgel, Arnaldo Correia de Medeiros, Eutília Andrade Medeiros Freire

ABSTRACT

Fibromuscular dysplasia (FMD) involves small- and medium-sized arteries, being a well-known cause of hypertension in young Caucasian women, when renal arteries are involved. The etiology of FMD remains unknown, despite many theories. A genetic component is suspected to exist, because the pathology affects primarily Caucasians. Association between FMD and the HLA-DRw6 histocompatibility antigen has also been described. The major sites affected are renal, cerebral, carotid, visceral, iliac, subclavian, brachial and popliteal arteries. Clinical manifestations correlate with the affected site, arterial hypertension being a frequent symptom, resulting from the involvement of the renal arteries in 60%-75% of the cases. The diagnosis of FMD is made by histopathology and/or angiography. 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. Rheumatologists should be aware of this differential diagnosis. Treatment of FMD is recommended only in symptomatic cases, and consists in revascularization, which may be either surgical or via percutaneous transluminal angioplasty. In FMD, the effects of corticotherapy can directly and rapidly harm the vascular wall, aggravating the lesions. More... »

PAGES

70-74

Identifiers

URI

http://scigraph.springernature.com/pub.10.1590/s0482-50042012000100008

DOI

http://dx.doi.org/10.1590/s0482-50042012000100008

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1043764754

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/22286647


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