Displasia fibromuscular en la Argentina. Registro del grupo de trabajo de Hipertensión Secundaria de la Sociedad Argentina de Hipertensión Arterial (SAHARA-DF): proyecto de protocolo y resultados preliminares / Fibromuscular dysplasia in Argentina. A registry from the Secondary Hypertension working group of the Argentine Society of Hypertension (SAHARA-DF): project protocol and preliminary results

En la Argentina no existen datos epidemiológicos sobre displasia fibromuscular. La realización de un registro nacional puede aportar información que conduzca a una actualización de los consensos y recomendaciones para un correcto diagnóstico, evaluación y tratamiento. El Registro Argentino de Displasia Fibromuscular (SAHARA-DF) inició su actividad de recopilación de datos en octubre de 2015. Al año 2019 se confirmaron 49 pacientes (44 mujeres, 38 hipertensos, edad 45,3 ± 17,2 años, 12 con presentación neurológica). Veintidós pacientes tuvieron lesiones vasculares en más de un sitio, a pesar del sesgo diagnóstico por falta de estudios complementarios en casi la mitad de los casos. El sitio afectado más frecuente fue el renovascular, seguido por el carotídeo y el ilíaco, y las lesiones multifocales fueron más frecuentes que las unifocales (35 versus 14, respectivamente). Se constató la presencia de aneurismas asociados en 13 casos y disección arterial en 4 casos. De las 22 angioplastias renales realizadas, 14 fueron con colocación de stent (endoprótesis). En este estudio preliminar de una población argentina se evidencia el carácter sistémico de la enfermedad y se plantea un llamado a actuar en cuanto a la necesidad de debatir el algoritmo diagnóstico y el método de tratamiento. (AU)

In Argentina there are no epidemiological data regarding fibromuscular dysplasia. Building a National Registry may provide information leading to updated consensus and recommendations for a correct diagnosis, assessment and treatment. Data gathering for the Argentine Registry of Fibromuscular Dysplasia (SAHARA-DF) was initiated in October 2015. By 2019, 49 patients were confirmed (44 women, 38 hypertensives, age 45.3 ± 17.2 years, 12 with a neurological presentation). Twenty-two patients had multi-site vascular lesions, in spite of a diagnosis bias due to lack of supporting studies in almost half of the cases. The renovascular site was the most affected, followed by the carotid and iliac sites, and multifocal lesions were more frequent than unifocal (35 versus 14, respectively). Associated aneurysms were found in 13 cases, and arterial dissection in 4. Twenty-two renal angioplasties were performed, 14 with stent placement. In this preliminary study of an Argentinian population, the systemic nature of the disease is evidenced, and a call for action arises regarding the need for discussing the diagnostic algorithm and treatment method. (AU)

A unique case of renal artery fibromuscular dysplasia.

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disorder resulting in arterial stenosis. Here, a unique case of renal artery FMD (RA FMD) is presented. With the use of angiograpic and intravascular ultrasound (IVUS) imaging modalities, a patient was diagnosed with two distinct types of FMD. Until now, the prevalence of FMD has been underestimated due to limitations in diagnostic modalities. Clinically, the correct identification of RA FMD is essential because these patients have been shown to respond to revascularization therapy with significant improvement in blood pressure whereas renal artery lesions secondary to other etiologies such as atherosclerosis have yet to show the same consistent response. Further, the presence of two subtypes of FMD in a single patient may suggest the possibility of genetic factors that predispose certain vessels to certain subtypes of FMD.

[Renal artery fibromuscular dysplasia]. / Dysplasie des artères rénales chez l'adulte.

Fibromuscular dysplasia (FMD) encompasses a heterogeneous group of idiopathic, segmental, nonatherosclerotic diseases of the musculature of arterial walls, leading to the narrowing of small and medium-sized arteries. The most common locations of FMD are renal arteries and carotid arteries. The diagnosis of FMD is made on the "string of beads" appearance of the arteries. The French Health Authority recommends performing a CT scan or an MRA to assess the diagnosis of FMD. A recent meta-analysis showed the cure rates using current definitions of hypertension cure are only 36% and 54% after angioplasty and surgery, respectively.

Fibromuscular dysplasia of cervical and intracranial arteries.

Fibromuscular dysplasia is an uncommon, segmental, nonatherosclerotic arterial disease of unknown aetiology. The disease primarily affects women and involves intermediate-sized arteries in many areas of the body, including cervical and intracranial arteries. Although often asymptomatic, fibromuscular dysplasia can also be associated with spontaneous dissection, severe stenosis that compromises the distal circulation, or intracranial aneurysm, and is therefore responsible for cerebral ischaemia or subarachnoid haemorrhage. Fibromuscular dysplasia affects middle and distal portions of the internal carotid and vertebral arteries, and occasionally, intracranial arteries. Several pathological and angiographic patterns exist. The most frequent pathological type is medial fibromuscular dysplasia, which is associated with the 'string of beads' angiographic pattern. Unifocal lesions are less common and can be associated with several pathological subtypes. The pathophysiology of the disease is widely unknown. Fibromuscular dysplasia may in fact result from various causes and reflect a non-specific response to different insults. The poor knowledge of the natural history and the lack of randomised trials that compared the different treatment options do not allow any satisfactory judgement to be made regarding the need for or the efficacy of any treatment.

Fibromuscular dysplasia.

Fibromuscular dysplasia (FMD), formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include atherosclerotic stenoses and stenoses associated with vascular Ehlers-Danlos and Williams' syndromes, and type 1 neurofibromatosis. Management of cases with renovascular hypertension includes antihypertensive therapy, percutaneous angioplasty of severe stenoses, and reconstructive surgery in cases with complex FMD that extends to segmental arteries. The therapeutic options for securing ruptured intracerebral aneurysms are microvascular neurosurgical clipping and endovascular coiling. Stenosis progression in renal artery FMD is slow and rarely leads to ischemic renal failure.

[Fibromuscular dysplasia]. / Dysplasie fibromusculaire artérielle.

Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic and noninflammatory disease of the muscle layer of arterial walls that leads to stenosis of small- and medium-sized arteries. Fibromuscular dysplasia preferentially affects young women. Although it can affect every arterial tree, it most often touches the renal and internal carotid arteries. Renal fibromuscular dysplasia can cause hypertension by stenosis of the renal artery, most often seen on angiography as resembling a "pearl necklace". Cerebrovascular fibromuscular dysplasia becomes symptomatic when the arterial stenosis is tight and causes hypoperfusion, embolism, or thrombosis or when arterial dissection or rupture of the associated aneurysm occurs.

Displasia fibromuscular septal de la arteria carótida interna, asociada a lupus eritematoso sistémico / Septal fibromuscular dysplasia of the internal carotid artery, associated to systemic lupus erytematosus

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Multiple basilar artery trunk aneurysms associated with fibromuscular dysplasia.

An association between fibromuscular dysplasia (FMD) and intracranial aneurysms has been proposed but the occurrence of multiple aneurysms on the trunk of basilar artery (BA) associated with FMD has not been previously reported.

Hipertensión arterial por displasia fibromuscular en dos hermanas / Hypertension for fibromuscular dysplasia in two sisters

Presentamos a dos hermanas con hipertensión arterial descubierta cuando se encontraban embarazadas y producida en ambos casos por displasia fibromuscular de las arterias renales. Comentamos la infrecuente incidencia familiar de la misma y sus posibles causas y revisamos los procedimientos diagnósticos y terapéuticos utilizados (AU)

Fibromuscular disease of the renal artery: a new histopathologic classification.

To evaluate the relationship between clinical, radiologic, and histopathologic features in various types of renal artery fibromuscular disease, the records of 37 patients operated upon for a total of 44 lesions of the renal artery (30 unilateral and seven bilateral) were retrospectively reviewed. Of these, 36 had hypertension associated with stenosis or aneurysm of the trunk or branches of the renal artery; one had aneurysm of the renal artery without hypertension. The histopathologic study included 38 arterial segments retrieved from 33 patients and underscored the difficulties in using topographic criteria to classify lesions. While all arterial aneurysms were associated with involvement of the media, more than one layer of the artery was involved in 65.7% of cases. Topographic forms, such as the perimedial type, were difficult to classify. Based on the study results, it appears essential to distinguish patients with fibrosis and smooth muscular hyperplasia (type I: 19 patients, 23 arteries) from patients with isolated fibrosis (type II: 14 patients, 15 arteries). In group II, preoperative hypertension was more long-standing (p less than 0.04), more lesions were extended distally (p less than 0.05), and the probability of recovery from hypertension at 52 months follow-up was lower. We have distinguished two different evolutive stages of fibromuscular disease of the renal artery, one characterized by fibrosis with smooth muscle hyperplasia and the second, associated with more severe prognosis, characterized by isolated fibrosis.