Dissections artérielles cervicales multiples chez deux frères : dysplasie fibro-musculaire ou maladie du tissu conjonctif ?

Verdure, P.; Triquenot-Bagan, Aude; Perdu, Jérôme; Gérardin, Emmanuel; Laquerrière, Annie; Hannequin, Didier; Guyant‐Maréchal, Lucie

doi:10.1016/s0035-3787(08)74101-8

Cited by 1 publication

(4 citation statements)

References 7 publications

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“…[1][2][3][4] It affects predominantly Caucasian, thin women, aged between 15 and 50 years, 5 with no familial history of the disease. 1 The etiology of FMD remains unknown regardless of countless theories.…”

Section: Definition Etiology and Epidemiologymentioning

confidence: 99%

“…6 In addition to the fi ndings suggesting genetic and hormonal etiologies, FMD has been reported in association with coagulation disorders, such as mutation of factor V Leiden, presence of antiphospholipid antibodies, mechanical stress, 5 and smoking. 3 …”

Section: Definition Etiology and Epidemiologymentioning

confidence: 99%

“…3 When involving the renal arteries, however, FMD is a well-known cause of hypertension in young Caucasian women.…”

Section: Major Arteries Affectedmentioning

confidence: 99%

“…9 While injury to the intima is seen as an image of focal and concentric stenosis, that of the adventitia appears as tubular stenosis on angiography. 3,5 In addition to those patterns, the arterial wall can be either thinner, due to rupture of the internal elastic lamina, originating an aneurysm, or thickened by dysplastic lesions, originating stenosis, which occurs in 16%-38% of the cases with renal artery involvement. It is worth noting that, when stenosis occurs, occlusion is rarely complete.…”

Section: Diagnosismentioning

confidence: 99%

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Displasia fibromuscular: um diagnóstico diferencial para as vasculites

Pontes¹,

Rufino²,

Gurgel³

et al. 2012

Rev. Bras. Reumatol.

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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 diffi cult in face of an atypical clinical presentation or lack of histopathologic confi rmation. 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.

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