Displasia fibromuscular y síndrome de ligamento arcuato medio. Infrecuentesy simultáneos / Fibromuscular dysplasia and median arcuate ligament syndrome. Rare and simultaneous findings

Introducción: la displasia fibromuscular (DFM) es una patología poco frecuente de la capa muscular de las arterias. El síndrome de ligamento arcuato medio (SLAM) es una entidad infrecuente causada por la compresión extrínseca del tronco celíaco por el diafragma. Caso clínico: presentamos el caso de una mujer joven con DFM diagnosticada de afectación a nivel del tronco celíaco y de la arteria hepática común. Ante clínica de dolor abdominal, se solicita angio TC, que describe un SLAM asociado a la DFM. Se decide sección quirúrgica del ligamento arcuato y descompresión del tronco celíaco mediante abordaje robótico. Discusión: en ambas entidades la angiografía es el trataminto de referencia para el diagnóstico. El tratamiento de primera línea de la DFM es el endovascular mediante angioplastia, y del SLAM, el quirúrgico, seccionando el ligamento arcuato.(AU)

Introduction: fibromuscular dysplasia (FMD) is a rare disorder that affects the muscular layer of the arteries. The medianarcuate ligament syndrome (MALS) is also a rare disorder due to the extrinsic compression of the celiac trunk by thediaphragm.Case report: we report the case of a young woman with FMD and splachnic involvement of the celiac trunk and thecommon hepatic artery level. After presenting with abdominal pain, a CCTA was performed that revealed the presenceof FMD-related MALS. The surgical section of the arcuate ligament and decompression of celiac trunk were decided andperformed through robotic approach.Discussion: the gold standard for the diagnosis of both entities is angiography. However, while the first-line therapy ofFMD is endovascular, in the case MALS the best alternative is surgical treatment sectioning the arcuate ligament.(AU)

Spontaneous coronary artery dissection: A review for clinical and interventional cardiologists.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome, especially among young to middle-aged women with few traditional cardiovascular risk factors and low pretest probability for atherosclerotic coronary artery disease. Diagnosis by invasive coronary angiography is the gold standard and conservative therapy is generally recommended, with percutaneous or surgical revascularization being reserved for cases of clinical instability, high-risk anatomy or as bailout. Unlike atherothrombotic coronary artery disease, strong evidence on optimal medical therapy is scarce, posing unique challenges in cases of pregnancy-associated SCAD. The follow-up strategy is also of major importance, as recurrent SCAD is not infrequent, lifestyle changes and pharmacological therapy should be planned for the long term, and SCAD-associated conditions need to be addressed. This review aims to provide a practical management approach to SCAD patients for both clinical and interventional cardiologists.

Neurofibromatosis type 1, fibromuscular dysplasia, and ischemic stroke: an association lost in time? A case report / Neurofibromatose tipo 1, displasia fibromuscular e acidente vascular cerebral isquêmico: uma associação perdida no tempo? Um relato de caso

Abstract Neurofibromatosis Type 1 (NF1) is a rare cause of ischemic stroke (IS) in the general population. We report a case of a young patient with NF1 in whom IS was caused by fibromuscular dysplasia. An angiographic study demonstrated occlusion in the right internal carotid artery (ICA), just after its origin, and the left ICA, just before the intracranial portion, and brain magnetic resonance imaging showed the limits of an area of brain infarction in the right frontoparietal region. Despite these concomitant neuroimaging findings, this association is rare, and it is difficult to establish the contribution to the outcome made by each of these diseases, which treatment is the best to implement, or what prognosis is.

Resumo A neurofibromatose tipo 1 (NF1) é uma causa rara de acidente vascular cerebral isquêmico (AVCi) na população geral. Neste estudo, relatamos o caso de um paciente jovem com AVCi, com diagnóstico de NF1 associada a displasia fibromuscular. O estudo angiográfico demonstrou oclusão da carótida interna direita, logo após sua origem, e esquerda, antes da porção intracraniana. A ressonância magnética do encéfalo mostrou delimitação de um infarto na região frontoparietal direita. Apesar desses achados concomitantes na neuroimagem, essa associação é rara, sendo difícil de estabelecer a contribuição de cada uma dessas doenças no desfecho, tampouco qual o melhor tratamento a ser implementado e qual o prognóstico.

Diseño y justificación del estudio clínico aleatorizado BA-SCAD (bloqueadores beta y agentes antiplaquetarios en pacientes con disección arterial coronaria espontánea) / Rationale and design of the BA-SCAD (Beta-blockers and Antiplatelet agents in patients with Spontaneous Coronary Artery Dissection) randomized clinical trial

Introducción y objetivos: La disección coronaria espontánea (DCE) es una causa rara de síndrome coronario agudo. La mayor parte de los pacientes con DCE son tratados empíricamente con bloqueadores beta (BB) y antiagregantes plaquetarios (AP). El estudio BA-SCAD (bloqueadores beta y agentes antiplaquetarios en pacientes con disección coronaria espontánea) es un ensayo clínico académico, pragmático, diseñado con metodología PROBE (prospective randomized open blinded endpoint), con el patrocinio de la Sociedad Española de Cardiología, para conocer la eficacia del tratamiento farmacológico en pacientes con DCE. Métodos: Mediante un diseño factorial 2 × 2, se aleatorizará a 600 pacientes (1:1/1:1) a: a) BB (sí/no) y b) tratamiento con AP «corto» (1 mes) frente a tratamiento antiagregante plaquetario doble y «prolongado» (12 meses). Se aleatorizará a BB (sí/no) solo a los pacientes con fracción de eyección del ventrículo izquierdo conservada, ya que a los pacientes con fracción de eyección reducida se los tratará con BB de acuerdo con las guías actuales. De modo similar, se aleatorizará al estrato de AP solo a los pacientes en tratamiento conservador (sin revascularización), ya que los que requieran intervención coronaria recibirán tratamiento antiagregante plaquetario doble durante 1 año. El objetivo primario de valoración incluye muerte, infarto de miocardio, accidente cerebrovascular, revascularización coronaria, DCE recurrente y hospitalización no planeada por síndrome coronario agudo o insuficiencia cardiaca al año de seguimiento. El objetivo de seguridad es la hemorragia. Todos los pacientes serán seguidos anualmente. Se desarrollará un programa exhaustivo de subestudios adicionales (clínicos, de imagen, de revascularización, de biomarcadores, inflamatorios, inmunológicos, farmacogenéticos y genéticos) para garantizar una visión completa de esta entidad tan especial y compleja (AU)

introduction y objectives: Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome. Most patients are empirically treated with beta-blockers and antiplatelet drugs. The Beta-blockers and Antiplatelet agents in patients with Spontaneous Coronary Artery Dissection (BA-SCAD) is an academic, pragmatic, prospective, randomized, open-label, blinded-endpoint clinical trial, performed under the auspices of the Spanish Society of Cardiology, to assess the efficacy of pharmacological therapy in patients with SCAD. Methods: Using a 2 x 2 factorial design, 600 patients will be randomized (1:1/1:1) to: a) beta-blockers (yes/no) and b) “short” (1 month) vs “prolonged” (12 months) antiplatelet therapy. Only patients with preserved left ventricular ejection fraction will be randomized to beta-blockers (yes/no) because patients with reduced left ventricular ejection fraction will receive beta-blockers according to current guidelines. Similarly, only conservatively managed patients (ie, no coronary intervention) will be randomized to the antiplatelet stratum, as patients requiring coronary interventions will receive 1-year dual antiplatelet therapy. The primary efficacy endpoint includes a composite of death, myocardial infarction, stroke, coronary revascularization, recurrent SCAD, and unplanned hospitalization for acute coronary syndrome or heart failure at 1 year. The primary safety endpoint will be bleeding. All patients will be clinically followed up yearly. A comprehensive set of additional substudies (clinical, imaging, revascularization, biomarkers, inflammatory, immunologic, pharmacogenetics, and genetic) will be conducted to ensure a holistic view of this unique and challenging clinical entity.Conclusions: The results of the BA-SCAD randomized clinical trial will advance our knowledge in the treatment of patients with SCAD (AU)

Rationale and design of the BA-SCAD (Beta-blockers and Antiplatelet agents in patients with Spontaneous Coronary Artery Dissection) randomized clinical trial.

INTRODUCTION Y OBJECTIVES: Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome. Most patients are empirically treated with beta-blockers and antiplatelet drugs. The Beta-blockers and Antiplatelet agents in patients with Spontaneous Coronary Artery Dissection (BA-SCAD) is an academic, pragmatic, prospective, randomized, open-label, blinded-endpoint clinical trial, performed under the auspices of the Spanish Society of Cardiology, to assess the efficacy of pharmacological therapy in patients with SCAD. METHODS: Using a 2 x 2 factorial design, 600 patients will be randomized (1:1/1:1) to: a) beta-blockers (yes/no) and b) "short" (1 month) vs "prolonged" (12 months) antiplatelet therapy. Only patients with preserved left ventricular ejection fraction will be randomized to beta-blockers (yes/no) because patients with reduced left ventricular ejection fraction will receive beta-blockers according to current guidelines. Similarly, only conservatively managed patients (ie, no coronary intervention) will be randomized to the antiplatelet stratum, as patients requiring coronary interventions will receive 1-year dual antiplatelet therapy. The primary efficacy endpoint includes a composite of death, myocardial infarction, stroke, coronary revascularization, recurrent SCAD, and unplanned hospitalization for acute coronary syndrome or heart failure at 1 year. The primary safety endpoint will be bleeding. All patients will be clinically followed up yearly. A comprehensive set of additional substudies (clinical, imaging, revascularization, biomarkers, inflammatory, immunologic, pharmacogenetics, and genetic) will be conducted to ensure a holistic view of this unique and challenging clinical entity. CONCLUSIONS: The results of the BA-SCAD randomized clinical trial will advance our knowledge in the treatment of patients with SCAD. The study was registered at ClinicalTrials.gov (Identifier: NCT04850417).

Spontaneous coronary artery dissection.

Spontaneous coronary artery dissection is an underdiagnosed entity. It has been recognized as an important cause of acute coronary syndrome and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. This document provides an exhaustive review of the pathophysiology and etiology of this disease. In addition, we describe the main clinical characteristics of these patients, the diagnostic tests that must be performed, and the most appropriate treatment.

Disección coronaria espontánea / Spontaneous coronary artery dissection

La disección coronaria espontánea es una entidad infradiagnosticada, que ha resultado ser una causa importante de síndrome coronario agudo y muerte súbita, en particular, en mujeres jóvenes y personas con pocos factores de riesgo cardiovascular convencionales. Este documento ofrece una revisión actualizada sobre la fisiopatología y etiología de esta enfermedad; se describen las principales características clínicas de estos pacientes, así como las pruebas diagnósticas que se deben realizar y el tratamiento más adecuado (AU)

Spontaneous coronary artery dissection is an underdiagnosed entity. It has been recognized as an important cause of acute coronary syndrome and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. This document provides an exhaustive review of the pathophysiology and etiology of this disease. In addition, we describe the main clinical characteristics of these patients, the diagnostic tests that must be performed, and the most appropriate treatment (AU)

Spontaneous coronary artery dissection. / Disección coronaria espontánea.

Spontaneous coronary artery dissection is an underdiagnosed entity. It has been recognized as an important cause of acute coronary syndrome and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. This document provides an exhaustive review of the pathophysiology and etiology of this disease. In addition, we describe the main clinical characteristics of these patients, the diagnostic tests that must be performed, and the most appropriate treatment.

Symptomatic internal carotid artery dissection and kinking in a patient with fibromuscular dysplasia / Dissecção e acotovelamento sintomáticos da artéria carótida interna em paciente com displasia fibromuscular

Abstract Isolated dissection of the internal carotid artery (ICA) is rare in young patients and is a cause for strong suspicion of fibromuscular dysplasia (FMD), especially when associated with artery elongation and tortuosity. The natural history of cerebrovascular FMD is unknown and management of symptomatic patients can be challenging. We report the case of a 44-year-old female patient with a history of transient ischemic attack in the absence of cardiovascular risk factors, associated with an isolated left ICA dissection and kinking. Carotid duplex ultrasound confirmed the diagnosis of dissection and demonstrated severe stenosis of the left ICA. The patient underwent surgical repair and histopathological evaluation confirmed the diagnosis of FMD with dissection. An autogenous great saphenous vein bypass was performed and the patient had an uneventful recovery. Cervical carotid artery dissection can be related to underlying arterial pathologies such as FMD, and the presence of ICA tortuosity highlights certain peculiarities for optimal management, which might be surgical.

Resumo A dissecção isolada da artéria carótida interna em pacientes jovens é rara, e a displasia fibromuscular deve ser altamente suspeitada principalmente quando estiver associada a alongamento e tortuosidade da artéria. A história natural da displasia fibromuscular cerebrovascular é desconhecida, e o manejo de pacientes sintomáticos pode ser desafiador. Apresentamos o caso de uma paciente de 44 anos com histórico de ataque isquêmico transitório sem fatores de risco cardiovasculares, associado a dissecção e acotovelamento isolados da artéria carótida interna esquerda. O ultrassom duplo das carótidas confirmou o diagnóstico de dissecção e demonstrou estenose grave na artéria carótida interna esquerda. A paciente foi submetida a reparo cirúrgico, e a avaliação histopatológica confirmou o diagnóstico de displasia fibromuscular com dissecção. Foi realizada cirurgia de ressecção do segmento e reconstrução com veia safena magna autógena, e a paciente se recuperou sem complicações. A dissecção da artéria carótida cervical pode estar relacionada a doenças arteriais subjacentes, como a displasia fibromuscular, e a presença da tortuosidade da artéria carótida interna destaca algumas particularidades no manejo ideal, o qual pode ser cirúrgico.

Carotid web: septo o banda carotídea: presentación de dos casos / Carotid web: "septo o banda carotidea": Two case reports

RESUMEN Carotid web es un término usado para describir en imágenes una anomalía vascular del desarrollo del sistema braquiocefálico. Se trata de una causa poco reconocida de accidente cerebrovascular, especialmente en pacientes jóvenes. Su frecuencia en la población general no se ha determinado y se asocia con eventos cerebrovasculares isquémicos de tipo embólico. En este reporte describimos dos casos de web carotídeo sintomático, uno que se manifiesta como un ataque isquémico transitorio (AIT) y el otro como infarto cerebral. Hasta el momento, no existe un método diagnóstico establecido como óptimo; la angiografía por resonancia magnética y por tomo-grafía se presentan como una opción confiable y alternativa a la angiografía convencional. Una vez identificado el carotid web, la evidencia es escasa con respecto a su manejo; algunas opciones con las que contamos son antiagregación, anticoagulación y manejo invasivo con endarterectomía y colocación de stent. Se dispone de poca literatura en español sobre web carotídeo. A efectos de una mejor comprensión proponemos el término de septo o banda carotídea para denominar a esta anomalía vascular.

SUMMARY Carotid web is a term used to describe by images a vascular abnormality of development of the brachiocephalic system. It is an under-recognized cause of stroke, especially in young patients. Its frequency in the general population has not been determined and is associated with ischemic cerebrovascular events of embolic type. Two cases of symptomatic carotid web are reported, one manifested as a transient ischemic attack (TIA) and the other as a cerebral infarction. There is not an established optimal diagnostic method, MRI and CT angiography are presented as a good option and alternative to conventional angiography. Once the carotid web has been identified, the evidence is scarce regarding its management, there are some options such as antiplatelets, anticoagulation and invasive management with endarterectomy and stent placement, there is little information in Spanish literature on carotid web so we propose the term septo o banda carotidea to name this vascular abnormality.

Infarto renal y disección carotídea espontánea: complicaciones de la displasia fibromuscular / Spontaneous renal infarction and carotid dissection: complications of fibromuscular dysplasia

Resumen La displasia fibromuscular es una enfermedad no inflamatoria y no aterosclerótica, que puede afectar cualquier lecho arterial; sin embargo, las arterias renales y la carótida interna son las más comprometidas. Se presenta el caso de un paciente que cursó con dolor abdominal y cefalea hemicraneana posterior, con síndrome de Horner incompleto. En arteriografía renal y panangiografía cerebral realizada por Cardiología intervencionista se observó infarto renal derecho y disección de la carótida interna derecha con formación de pseudoaneurisma. Se hizo terapia endovascular con angioplastia y se dio de alta con antiagregación dual. Es importante conocer este tipo de presentación clínica ya que el diagnóstico podría confundirse con otras enfermedades que generan signos y síntomas similares; por consiguiente, es ideal tener sospecha clínica alta para evitar retrasos en el manejo.

Abstract Fibromuscular dysplasia is a non-inflammatory and non-atherosclerotic disease that can affect any arterial bed, with the renal and the internal carotid arteries being the most compromised. A case is presented on a patient that had abdominal pain and pain in the back of the head, and an incomplete Horner syndrome. In the renal arteriography and cerebral pan-angiography carried out by interventionist Cardiology, a right renal infarction and dissection of the right internal carotid was observed with a pseudo-aneurysm formation. Intravenous treatment was performed with angioplasty, and the patient was discharged with dual antiplatelet therapy. It is important to be aware of this type of clinical presentation, since the diagnosis can be confused with other diseases that produce similar signs and symptoms. It would be ideal to have a high clinical suspicion in order to avoid delays in the management.

[Bilateral renal infarction: A rare complication of fibromuscular dysplasia]. / Infarto renal bilateral: una rara complicación de la displasia fibromuscular.

Bilateral renal infarction complicating Fibromuscular Dysplasia (FMD) is extremely rare and has so far been reported only in a handful of cases. We report a 44-year-old man who presented with complaints of abdominal pain and severe hypertension. Computed tomography of the abdomen revealed bilateral renal infarction and a renal angiography confirmed the bilateral and irregular stenosis due to FMD, in the absence of systemic vasculitis and other risk factors for thromboembolic events. Therefore, angioplasty with balloon was performed in order to obtain a good vessel patency and to improve patency in the long term follow-up. After the endovascular treatment the blood pressure improved markedly, maintaining this result at 3-months follow-up. Our case highlight the importance of recognizing renal infarction as an initial presentation of FMD.

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)

Estenosis bilateral de arteria renal por displasia fibromuscular / Renal artery stenosis due to fibromuscular dysplasia

Resumen La estenosis de la arteria renal es una afección clínica que puede ser asintomática o causar hipertensión arterial sistémica, así como deterioro de la función renal de uno o ambos riñones. La estenosis puede ser causada por aterosclerosis en 90% de los casos y por displasia fibromuscular en 10%, la estenosis de la arteria renal es la principal causa de hipertensión secundaria reversible o modificable si ésta es diagnosticada y tratada antes de cursar con el daño renal. La displasia fibromuscular es indicación de angioplastia percutánea logrando el control de la presión arterial en, incluso, 63% sin antihipertensivos. El objetivo de este artículo es mostrar la complejidad del proceso diagnóstico y la importancia que tiene la sospecha clínica para la corrección oportuna de este tipo de hipertensión arterial reversible.

Abstract Renal artery stenosis (RAS) is a clinical entity that can be asymptomatic or cause systemic arterial hypertension likewise spoil the renal function of one or both kidneys. The stenosis can be caused by atherosclerosis in 90% of the cases and by fibromuscular dysplasia in 10%; the stenosis of the renal artery is the main cause of reversible or modifiable secondary hypertension if it is diagnosed and treated before the renal damage. Fibromuscular dysplasia is an indication for percutaneous angioplasty achieving blood pressure control in up to 63% without antihypertensive drugs. The objective of this paper is to show the complexity of the diagnostic process and the importance of the clinical suspicion for the well-timed correction of this type of arterial hypertension.

Abordagem endovascular de paciente com fibrodisplasia de artéria renal bilateral associada a volumoso aneurisma renal / Endovascular approach of a patient with bilateral renal artery fibrodysplasia associated with a massive renal aneurysm

A fibrodisplasia muscular renal é de etiologia desconhecida, de origem não inflamatória e não aterosclerótica, estando associada ao desenvolvimento de estenoses e de aneurismas. Relatamos um caso de fibrodisplasia muscular renal bilateral associada a aneurisma volumoso de artéria renal tratado com angioplastia com balão e stent multicamadas.

Renal artery fibromuscular dysplasia is a condition of unknown etiology, with non-inflammatory, nonatheroscleroticorigin, associated to the development of stenosis and aneurysms. The authors report a case of bilateral renal artery fibromuscular dysplasia associated with a large renal artery aneurysm, treatedwith balloon angioplasty and a multilayer stent.

Hipertensión arterial secundaria a displasia fibromuscular de la arteria renal. A propósito de un caso / Hypertension secondary to fibromuscular dysplasia of the renal artery. Report of a case

Se presenta el caso clínico de una paciente que debutó con hipertensión arterial (HTA) grado II realizándose los estudios sistematizados correspondientes para descartar patologías secundarias. Dentro de los estudios de primera línea en la valoración merece destacarse la ecografía Doppler renal por la información que ella aporta, orientado al diagnóstico de estenosis de la arteria renal. La identificación de una HTA de origen renovascular supone el hallazgo de una de las causas reversibles de hipertensión. Dentro de esta etiología la más frecuente es la displasia fibromuscular (DFM) que mejora con la corrección de la estenosis mediante técnicas quirúrgicas o percutáneas con angioplastia con balón o stent. El objetivo es presentar un caso clínico para mostrar la complejidad del proceso diagnóstico y la importancia que tiene la sospecha clínica para la corrección oportuna de este tipo de HTA reversible...

Displasia fibromuscular: um diagnóstico diferencial para as vasculites / Fibromuscular dysplasia: a differential diagnosis of vasculitis

A displasia fibromuscular (DFM) envolve artérias de pequeno e médio calibre e é uma causa bem conhecida de hipertensão em mulheres jovens caucasianas, quando envolve as artérias renais. A etiologia da DFM permanece desconhecida, a despeito de inúmeras teorias. Há suspeita de um componente genético, já que a doença atinge primariamente caucasianos. Também é descrita associação entre DFM e antígeno de histocompatibilidade HlA-DRw6. Os principais sítios acometidos são as artérias renais, cerebrais, carótidas, viscerais, ilíacas, subclávias, braquiais e poplíteas. As manifestações clínicas correlacionam-se com o sítio acometido, e a hipertensão arterial sistêmica é um sintoma frequente pelo acometimento das artérias renais em 60 por cento-75 por cento dos casos. O diagnóstico da DFM é feito por meio de exame histopatológico e/ou angiográfico. A DFM pode apresentar-se como doença vascular sistêmica, mimetizando vasculites. Essa compreensão é importante porque tanto a vasculite quanto a DFM podem ter curso clínico grave, e exigem tratamentos diferentes. O diagnóstico diferencial pode ser difícil diante de uma apresentação clínica atípica ou da incapacidade de obter a confirmação histopatológica. Há relatos isolados de DFM mimetizando poliarterite nodosa, síndrome de Ehlers-Danlos, síndrome de Alport, feocromocitoma, síndrome de Marfan e arterite de Takayasu. Os reumatologistas devem estar atentos para fazer esse diagnóstico diferencial. O tratamento da DFM é recomendado apenas em casos sintomáticos, e consiste em revascularização (cirúrgica ou por angioplastia percutânea transluminal). Na DFM, efeitos da corticoterapia podem ser direta e rapidamente deletérios para a parede vascular, levando à piora das lesões.

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 percent-75 percent 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.