OBJECTIVE: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet\'s syndrome (BS) (difficult to treat, severe resistant, severe relapse) to conventional treatment.
METHODS: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format (patient, intervention, comparison and outcome). Systematic reviews of the evidence were conducted, the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations Assessment, Development, and Evaluation (GRADE). After that, the multidisciplinary panel formulated the specific recommendations.
RESULTS: 4 PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with BS with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus.
CONCLUSIONS: The treatment of most severe clinical manifestations of BS lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official Recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.

BACKGROUND: Ascending aortic aneurysms are rare pathologies in childhood, especially in the absence of previous diseases such as Marfan syndrome.
OBJECTIVE: Present the possibility of successful endovascular management of large vessel aneurysms, using stents and microcatheters with embolization of the aneurysm sac.
METHODS: We present the case of a previously healthy ten-year-old patient, in whom a pseudoaneurysm was documented between the origin of the left common carotid artery and left subclavian artery, successfully managed endovascularly, initially with a stent covering the neck of the aneurysm to remodel it and later with embolization of the aneurysm sac using a microcatheter.
RESULTS: Aneurysms of large vessels, such common carotid artery and subclavian artery, are at risk of rupture with devastating complications; endovascular management is considered a minimally invasive management option, with favorable results.
CONCLUSIONS: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.
BACKGROUND: Los aneurismas de la aorta ascendente son patologías poco frecuentes en la infancia, sobre todo en ausencia de enfermedades previas como el síndrome de Marfan.
OBJECTIVE: Dar a conocer la posibilidad del manejo endovascular exitoso de los aneurismas de grandes vasos, usando stent y micro catéter con embolización del saco aneurismático.
UNASSIGNED: Presentamos el caso de una paciente de 10 años y 2 meses, previamente sana, en quien se documentó un pseudoaneurisma entre el origen de la arteria carótida común izquierda y la arteria subclavia izquierda, que logró manejarse de forma endovascular, inicialmente con un stent cubriendo el cuello del aneurisma con el fin de remodelarlo y posteriormente por medio de microcatéter se realizó embolización del saco del aneurisma con coils, con resultado exitoso.
RESULTS: Los aneurismas de los grandes vasos, como la arteria carótida común y la arteria subclavia, tienen riesgo de ruptura con complicaciones devastadoras; el manejo endovascular se plantea como una opción poco invasiva de manejo, con resultados favorables.
UNASSIGNED: El manejo de aneurismas de grandes vasos, por vía endovascular usando stent y microcatéter con embolización del saco aneurismático, es una opción novedosa de manejo que logra resultados exitosos.

In spontaneous subarachnoid haemorrhage (SAH) accurate determination of the bleeding source is paramount to guide treatment. Traditionally, the bleeding pattern has been used to predict the aneurysm location. Here, we have tested a software-based tool, which quantifies the volume of intracranial blood and stratifies it according to the regional distribution, to predict the location of the ruptured aneurysm.
A consecutive series of SAH patients admitted to a single tertiary centre between 2012-2018, within 72 h of onset, harbouring a single intracranial aneurysm. A semi-automatized method of blood quantification, based on the relative density increase, was applied to initial non-contrast CTs. Five regions were used to define the bleeding patterns and to correlate them with aneurysm location: perimesencephalic, interhemispheric, right/left hemisphere and intraventricular.
68 patients were included for analysis. There was a strong association between the distribution of blood and the aneurysm location (p < 0.001). In particular: ACom and interhemispheric fissure (p < 0.001), MCA and ipsilateral hemisphere (p < 0.001), ICA and ipsilateral hemisphere and perimesencephalic cisterns (p < 0.001), PCom and hemispheric, perimesencephalic and intraventricular (p = 0.019), and PICA and perimesencephalic and intraventricular (p < 0.001). The internal diagnostic value was high (AUROC ≥ 0.900) for these locations.
Regional automatised volumetry seems a reliable and objective tool to quantify and describe the distribution of blood within the subarachnoid spaces. This tool accurately predicts the location of the ruptured aneurysm; its use may be prospectively considered in the emergency setting when speed and simplicity are attained.

We present a case of a ruptured right sinus of Valsalva aneurysm to the right atrium that developed global heart failure over the course of three months, and which was completely resolved through cardiac catheterism, placing an occlusive device at the site of the fistula. Its ethology is discussed, as well as the guidelines for clinical diagnosis and treatment.
Se presenta un caso de aneurisma del seno de Valsalva derecho roto a la aurícula derecha, que en el transcurso de tres meses desarrolló insuficiencia cardiaca global y fue resuelto del todo por medio de intervencionismo, colocando un dispositivo oclusor en el sitio de la fístula. Se discute su etología, así como las pautas para el diagnóstico clínico y el tratamiento.

Multiple scales have been designed to stratify the severity and predict the prognosis in the initial evaluation of patients with aneurysmal subarachnoid hemorrhage (aSAH). Our study aimed to validate the most commonly used prognostic scales for aSAH in our population: Hunt-Hess, modified Hunt-Hess, World Federation of Neurosurgical Societies (WFNS), Prognosis on Admission of Aneurysmal Subarachnoid Hemorrhage (PAASH), and Barrow Aneurysm Institute (BAI) scales.
This study includes all aSAH cases treated at our institution between June 2019 and December 2020. We developed a retrospective cohort by reviewing medical records and radiologic images performed during hospitalization. The outcome was evaluated using the modified Rankin scale (mRS). It was defined as a poor outcome (mRS 4-5) and mortality (mRS 6). The ROC curves and the area under the curve (AUC) of each of the prognostic scales were calculated to evaluate their prognostic prediction capacity.
A total of 142 patients were diagnosed with aSAH. A poor outcome occurred in 52.1% of the patients, whereas mortality was 27.5%. The AUC of the scales studied was similar and no significant difference was found between them for predicting a poor outcome (P = .709) or mortality (P = .715).
We determined that the prognostic scales for aSAH had a similar predictive value for poor clinical outcomes and mortality in our institution, with no significant difference. Thus, we recommend the most simple and well-known scale used institutionally.

BACKGROUND: Relatively constant surgical risks and rapid advances in endovascular treatment have caused a major shift toward endovascular management of posterior circulation aneurysms. This paper presents the results of a series of endovascularly treated posterior circulation aneurysms.
METHODS: A total of 81 patients who underwent endovascular treatment of posterior circulation aneurysms performed by a single team between 2009 and 2019 were included. Demographic, clinical, radiologic, and management details were retrospectively obtained from hospital records.
RESULTS: Among the included patients, 50 (61.7%) and 31 (38.3%) were female and male, respectively. Subarachnoid hemorrhage was observed in 30 patients (37%). Moreover, 40 (49.3%) aneurysms were treated with stent-assisted coiling, 1 (1.2%) aneurysm was treated with parent artery occlusion, 2 (2.4%) aneurysms were coiled using balloon assistance, 24 (29.6%) aneurysms were coiled primarily, 1 (1.2%) patient had an unsuccessful treatment attempt, and 13 (16.0%) aneurysms were treated with flow-diverter stents or stent monotherapy. During the last follow-up, 57 (83.8%) aneurysms were completely occluded, whereas 6 (8.8%) and 2 (2.9%) aneurysms did and did not have a residual neck, respectively. Flow diversion was used to treat 13 patients, among whom 8 had total occlusion or stable residue. A total of 7 deaths (8.6%) were encountered in this series.
CONCLUSIONS: Endovascular treatment should be considered as the primary treatment modality for posterior circulation aneurysms. Despite the high morbidity and mortality rates, promising results can be achieved with correct patient selection. Flow diversion can be a feasible alternative for complex aneurysms that are difficult to treat.

BACKGROUND: Upper extremity arterial aneurysms are not common clinical conditions. It may causes ischemic gangrene or limb losses as a result of thromboembolic events due to endothelial damage. In this study, we aimed to investigate the etiology, management, and long-term outcome.
METHODS: A total of 55 upper extremity aneurysms between January 2009 and April 2018 were retrospectively investigated. The mean age was 41 ± 13 years, and the women and men were 13 (23.6%) and 42 (76.4%). The mean follow-up was 43 ± 18 months.
RESULTS: The incidence of pseudoaneurysm was higher than the true aneurysm (64.5% vs. 35.5%). 30 (84%) patients who had pseudoaneurysm repaired primerely and 6 (16%) patients had patchplasty. About 58% of the true aneurysm caused by blunt trauma. 4 (21%) true brachial aneurysm patients were required emergency intervention due to distal ischemia. 16 (84.2%) patients operated using by saphenous vein graft. The primary and secondary patency was 87.5% and 93.8%. No limb or life loss occurred during follow-up.
CONCLUSIONS: Pseudoaneurysms are more common among upper limb aneurysms and they occur mostly by iatrogenic causes. Blunt trauma can be main cause of the true aneurysm. Surgical resection of the aneurysm and interposition of saphenous vein graft provides excellent results in the long-term.
BACKGROUND: los aneurismas arteriales de las extremidades superiores no son condiciones clínicas frecuentes. Puede causar gangrena isquémica o pérdida de extremidades como resultado de eventos tromboembólicos por daño endotelial. En este estudio, nuestro objetivo fue investigar la etiología, el tratamiento y el resultado a largo plazo.
UNASSIGNED: Se investigaron retrospectivamente un total de 55 aneurismas de las extremidades superiores sometidos a reparación quirúrgica entre enero de 2009 y abril de 2018. La edad media fue de 41 ± 13 años, y las mujeres y los hombres tenían 13 (23.6%) y 42 (76,4%). El seguimiento medio fue de 43 ± 18 meses.
RESULTS: La incidencia de pseudoaneurisma fue mayor que el aneurisma verdadero (64.5% frente a 35.5%) y fue causado por un traumatismo iatrogénico. 30 (84%) de los pseudoaneurismas reparados con cualquier injerto, 6 (16%) pacientes requirieron plastia con parche. 58% del aneurisma verdadero causado por traumatismo cerrado. 4 (21%) pacientes con aneurisma braquial verdadero requirieron intervención de emergencia debido a isquemia distal. 16 (84,2%) pacientes fueron sometidos a operación de reparación de aneurisma mediante injerto de vena safena. La permeabilidad primaria y secundaria fue del 87.5% y 93.8%. No se produjeron pérdidas de miembros ni de la vida durante el seguimiento.
UNASSIGNED: Los pseudoaneurismas son más comunes entre los aneurismas de miembros superiores y ocurren principalmente por causas iatrogénicas. El traumatismo cerrado es la principal causa del verdadero aneurisma. La resección quirúrgica del aneurisma y la interposición con injerto de vena safena proporciona excelentes resultados a largo plazo.

Low back pain is one of the most frequent reasons for consultation in primary care. Sometimes it is not easy to make a good differential diagnosis, because the number of pathologies that can express themselves with such symptoms is very wide and a complementary test or referral to the reference hospital is not always indicated. The following is a clinical case of a 59-year-old female patient with a history of dyslipidemia treated with ezetimibe, who consulted again for breakthrough low back pain, which was finally diagnosed as a penetrating ulcer of the abdominal aorta, treated endovascularly by the vascular surgery service. The clinical case aims to provide an overview of the acute treatment of aortic syndrome, showing the differences compared to the management of other common pathologies in primary care with the same clinical expression.

to describe the implantation of ultrasound screening for Abdominal Aortic Aneurysm (AAA) in our healthcare district in men from 65 to 79 years of age who have had an identifiable risk factor for developing AAA, such as smoking or a history thereof, hypertension, family history of aneurysms, aneurysms in other locations and clinical atherosclerosis, acute myocardial infarction, intermittent claudication, or stroke. Analyse the performance of said screening.
Primary Care.
656 patients were screened, representing 40% of the target population of 1,658 patients. The remaining part of the target population could not be screened because of the outbreak of the COVID-19 pandemic. 608 ultrasound examinations were performed.
coverage of the screening programme, prevalence of abdominal aortic aneurysms, prevalence of smoking and other risk factors in patients with/without aneurysms.
19 patients with ectatic aorta (25-29mm) and 11 with abdominal aortic aneurysms (1.81%) were found. 5 were active smokers (45%, compared to 20% in the entire sample) and 6 were former smokers. None of the aneurysm patients were non-smokers. 7 of them were hypertensive.
The prevalence of aneurysms in our sample was 2.6%, which was lower than expected. The wide use of ultrasound and its progressive generalisation in the Primary Care setting should lead to a decrease in the number of undiagnosed AAA.

UNASSIGNED: In the cavum segment the internal carotid artery has more susceptibility formation of aneurysms. The Solitaire™ AB stent is a scaffold that do flow blood diversion and promotes the aneurysms exclusion by thrombosis.
UNASSIGNED: 63 year old female present pulsatile headache, diplopia, III left cranial nerve paresis and carried a cavum carotid saccular aneurysms of the left carotid artery. By endovascular procedure, we placed a Solitaire™ AB stent. She recovered the III left cranial nerve function in to 2 month and we observed exclusion of the aneurysms by DynaCT in 3 months.
UNASSIGNED: The reconstruction of an artery by endovascular therapy is an alternative treatment for no ruptured paraclinoid aneurysms.
UNASSIGNED: La arteria carótida interna a nivel del cavum es más susceptible de formación de aneurismas. El Solitaire™ AB condiciona una redirección del flujo de la sangre que favorece la exclusión por trombosis del aneurisma.
UNASSIGNED: Mujer de 63 años que presentó cefalea, diplopía, paresia del III nervio craneal y portadora de aneurisma sacular en la arteria carótida interna izquierda a nivel del cavum carotídeo. Se colocó una endoprótesis Solitaire™ AB por procedimiento endovascular. Recuperó la función del III nervio craneal izquierdo a los 2 meses y se observó la exclusión del aneurisma a los 3 meses por DynaCT.
UNASSIGNED: La endoprótesis para reconstrucción arterial intracraneal es una alternativa terapéutica en aneurismas paraclinoideos no rotos.