ABSTRACT
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)
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Young Adult , Records/statistics & numerical data , Fibromuscular Dysplasia/diagnosis , Argentina/epidemiology , Algorithms , Bias , Sex Factors , Cross-Sectional Studies , Risk Factors , Age Factors , Angioplasty/methods , Cultural Factors , Vascular System Injuries/diagnostic imaging , Fibromuscular Dysplasia/classification , Fibromuscular Dysplasia/etiology , Fibromuscular Dysplasia/therapy , Fibromuscular Dysplasia/epidemiology , Hypertension/epidemiology , Aortic Dissection/diagnostic imagingABSTRACT
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...
Subject(s)
Humans , Adolescent , Female , Fibromuscular Dysplasia/complications , Fibromuscular Dysplasia/diagnosis , Fibromuscular Dysplasia/therapy , Hypertension, Renovascular/etiology , Renal Artery Obstruction/therapy , Echocardiography, Doppler , Physical ExaminationABSTRACT
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.
Subject(s)
Female , Humans , Fibromuscular Dysplasia/diagnosis , Vasculitis/diagnosis , Diagnosis, DifferentialSubject(s)
Humans , Male , Adult , Angioplasty , Fibromuscular Dysplasia/surgery , Fibromuscular Dysplasia/diagnosis , Hypertension/complications , Renal Artery Obstruction/surgery , Renal Artery Obstruction/diagnosis , Angiography , Fibromuscular Dysplasia/etiology , Renal Artery Obstruction/etiology , Ultrasonography, DopplerABSTRACT
A case with renovascular hypertension due to fibromuscular dysplasia [FMD] was associated with cerebral aneurysm. This patient was a twelve-year-old girl with cerebral aneurysm and complete obstruction of the right renal artery. The patient's cerebral aneurysm was successfully treated by endovascular techniques with successful right side nephrectomy, and pathologic changes corresponded to FMD. After nephrectomy, the patient's blood pressure became normal with no need for medical therapy. This report suggests that FMD should be considered to be a systemic angiopathy, including the cerebral artery as well as the renal artery
Subject(s)
Humans , Female , Fibromuscular Dysplasia/diagnosis , Hypertension, Renovascular , Intracranial Aneurysm/etiology , Intracranial Aneurysm/therapy , Angioplasty , Renal Artery Obstruction , Nephrectomy , Blood Pressure , Vascular DiseasesABSTRACT
Displasia fibromuscular intracraniana isolada é rara e pode se apresentar como acidente vascular cerebral (AVC). Ela deve ser considerada como etiologia do AVC em paciente jovem saudável. Embora o seu diagnóstico possa ser um desafio, características morfológicas específicas podem ser reveladas através da angiografia através de ressonância nuclear magnética e por de catéter. A displasia fibromuscular envolvendo a circulação cérvico-cefálica afeta tipicamente a artéria carótida interna extracraniana (95%). O presente caso relata uma rara ocorrência de displasia fibromuscular intracraniana em uma jovem de 19 anos com infarto do núcleo caudado e joelho da cápsula interna à esquerda.
Subject(s)
Adult , Female , Humans , Anterior Cerebral Artery/pathology , Fibromuscular Dysplasia/complications , Stroke/etiology , Cerebral Angiography , Caudate Nucleus/pathology , Fibromuscular Dysplasia/diagnosis , Magnetic Resonance Angiography , Stroke/diagnosisABSTRACT
We report a 39 years old male presenting with an intense hemicrania pain in the tract of the carotid artery and Claude Bernard Horner syndrome. The patient had also a high blood pressure. Magnetic resonance imaging demonstrated a left carotid artery fibrodysplastic disease. Conventional angiography of 4 cerebral vessels showed a dissection of the left carotid artery and fibromuscular dysplasia of the left vertebral artery. Renal angiography showed a stenosis and occlusion of renal artery with radiological exclusion of right kidney. On the left side there was a stenosis and occlusion of segmental branches. The diagnostic importance of Claude Bernard Horner syndrome and the need to study renal artery involvement when carotid dissection is accompanied with high blood pressure, is highlighted
Subject(s)
Humans , Male , Adult , Renal Artery , Fibromuscular Dysplasia/diagnosis , Carotid Artery, Internal , Atenolol/therapeutic use , Ticlopidine/therapeutic use , Angiography , Carotid Artery Diseases , Doxazosin/therapeutic use , Fibromuscular Dysplasia/complications , Migraine Disorders/etiology , Hypertension/complications , Hypertension/drug therapy , Horner Syndrome/complicationsABSTRACT
Arrthythmogenic right ventricular dysplasia is a form of cardiomyopathy which causes life threatening ventricular tachyarrhythmias in adults. We present a cases who ultimately expired along with review of literature of this entity
Subject(s)
Humans , Male , Fibromuscular Dysplasia/diagnosisABSTRACT
Relatos de displasia fibromuscular da artéria basilar säo raros, só sendo encontrados após revisäo da literatura em três publicaçöes. Teve-se a oportunidade de acompanhar um quarto caso, no qual a lesäo constituiu achado angiográfico em paciente com encefalopatia hipertensiva. Apresenta-se este caso, associado à discussäo sobre os aspectos mais relevantes nesta condiçäo
Subject(s)
Adult , Humans , Female , Basilar Artery , Fibromuscular Dysplasia/diagnosis , AngiographyABSTRACT
Se describen tres casos de displasia arterial en mujeres jovenes afectando la arteria renal, la poplitea y las coronarias, respectivamente. En todos se observa el tipico cambio de disrupcion de la arquitectura de la media con cambios variables en la intima y la adventicia. Cuando la lesion es de la arteria renal se produce hipertension; al afectarse otras arterias el resultado es la insuficiencia vascular correspondiente. La displasia arterial se puede diferenciar por sus caracteristicas histologicas de otras enfermedades vasculares. La etiologia es desconocida