Is Fibromuscular Dysplasia a Single Disease?

Olin, Jeffrey W.

doi:10.1161/circulationaha.112.149500

Cited by 27 publications

(30 citation statements)

References 21 publications

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“…6 Because all patients in this series presented with hypertension and renal artery FMD, it is not clear whether this phenotypic difference will also be present in those with FMD in other arterial locations. 59 Acknowledging the practicality and appropriateness of an angiographic classification, we propose an American Heart Association classification that is a minor modification in the classification proposed by the European Consensus (Tables 1 and 2). 2 Multifocal disease is the classic string of beads appearance represented by medial fibroplasia in virtually all adults.…”

Section: Histopathological Classification Systems For Fmdmentioning

confidence: 99%

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Olin¹,

Gornik²,

Bacharach³

et al. 2014

Circulation

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Section: Histopathological Classification Systems For Fmdmentioning

confidence: 99%

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Olin¹,

Gornik²,

Bacharach³

et al. 2014

Circulation

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395

490

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“…The arterial manifestations of FMD include the "string-of-beads" appearance in multifocal disease, focal stenosis in focal FMD, as well as aneurysm, dissection, and arterial tortuosity [3][4][5][6][7]. These findings have been described in virtually every arterial bed.…”

Section: Introductionmentioning

confidence: 99%

“…Since the advent of endovascular therapy, tissue for the histologic diagnosis of FMD is rarely available; therefore, FMD is an imaging diagnosis. Dissection, aneurysm, and/or tortuosity can only be attributed to FMD if the focal or multifocal findings are identified in a separate arterial bed [4,5]. The most common locations of disease are the carotid and renal arteries, and clinical manifestations reflect the affected circulation [8].…”

Section: Introductionmentioning

confidence: 99%

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Kadian-Dodov¹,

Goldfinger²,

Gustavson³

et al. 2018

Cerebrovasc Dis

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Background and Purpose: Fibromuscular dysplasia (FMD) is a non-atherosclerotic arteriopathy most often affecting the carotid and renal arteries. In the United States Registry for FMD, 41.7% of patients experienced an aneurysm and/or dissection by the time of entry into the Registry. We sought to determine the occurrence of neurovascular events after FMD diagnosis and any changes on cervical artery imaging that may be attributable to FMD. Methods: Patients followed at the Mount Sinai Medical Center (US Registry for FMD enrollment center) with confirmed FMD and > 1 cervical artery imaging study (at least ± 6 months from the baseline carotid duplex ultrasound [CDU]) between the years 2003 and 2015 were included. Medical records and cervical artery imaging ([CDU], magnetic resonance angiogram [MRA], and computed tomography angiogram [CTA]) were reviewed. New arterial dissection, aneurysm, transient ischemic attack, stroke, or new FMD findings were recorded. Results: Among 146 FMD patients with complete information, 52 (35.6%) had an aneurysm and 52 (35.6%) had a dissection. Mean clinical follow-up was 35.3 ± 25.3 months (range 5–153 months); patients underwent 4 ± 2.7 CDU (range 1–17); 86.3% had ≥1 neck MRA or CTA. After FMD diagnosis, 3 patients (2%) experienced a new carotid artery dissection; 1 patient experienced a stroke due to concomitant atherosclerosis. No new aneurysms occurred. In patients with cervical artery FMD, imaging findings remained stable throughout follow-up. No patient developed new cervical artery FMD findings on follow-up imaging. Conclusions: No new cervical artery FMD or aneurysm was observed on subsequent imaging. New carotid dissection was uncommon over a mean follow-up period of 35.3 ± 25.3 months and was the only non-atherosclerotic vascular event observed after FMD diagnosis.

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“…Adventitial FMD is uncommon. However, few dysplasias are currently examined histologically, so an angiographic diagnostic classification has been suggested based on differentiation between unifocal and multifocal appearance 32. Unifocal FMD (Figure 2) has less of a female predominance and is diagnosed more often in younger individuals, with better short-term and long-term results from treatment than multifocal FMD (Figure 1).…”

Section: Pathologymentioning

confidence: 99%

“…Unifocal FMD (Figure 2) has less of a female predominance and is diagnosed more often in younger individuals, with better short-term and long-term results from treatment than multifocal FMD (Figure 1). 32…”

Section: Pathologymentioning

confidence: 99%

Optimal management of renal artery fibromuscular dysplasia

Gottsäter¹,

Lindblad²

2014

TCRM

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Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory angiopathy of unknown cause affecting medium-sized (most commonly renal) arteries and causing renovascular hypertension. The most common medial multifocal type of FMD (with the “string of beads” appearance) is more than four times more prevalent in females than in males. FMD accounts for up to 10% of cases of renovascular hypertension. Compared with patients with atherosclerotic renal artery stenosis, patients with FMD are younger, have fewer risk factors for atherosclerosis, and a lower occurrence of atherosclerosis in other vessels. The etiology is multifactorial, including vessel wall ischemia and smoking, as well as hormonal and genetic factors. Intra-arterial digital subtraction angiography is still the gold standard for exclusion or confirmation of renal artery stenosis caused by FMD, at least in young patients, who more often have lesions in branches of the renal artery. For FMD patients with atherosclerosis and those who are older (>50–55 years), significant renal artery stenosis may be confirmed or excluded with ultrasonography. The FMD lesion is typically truncal or distal, whereas atherosclerotic lesions are more often proximal or ostial. Treatment options are medical, endovascular (percutaneous transluminal renal angioplasty [PTRA]), and surgical. Invasive treatment should be considered when hypertension cannot be controlled with antihypertensive drugs and in patients with impaired renal function or ischemic nephropathy. PTRA has become the treatment of choice and normally yields good results, especially in unifocal disease and young patients. Pressure gradients are normally completely abolished, and there is no indication for stent placement. Surgical revascularization is indicated after PTRA complications; thrombosis, perforation, progressive dissection, repeated PTRA failure or restenosis. Centralization of handling is recommended.

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Is Fibromuscular Dysplasia a Single Disease?

Cited by 27 publications

References 21 publications

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Optimal management of renal artery fibromuscular dysplasia

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