Serum leukemia inhibitory factor (LIF) levels in patients with Takayasu's and Giant cell arteritis: A cross-sectional study.

INTRODUCTION AND OBJECTIVES: In this study, we aimed to evaluate LIF levels and its possible relationship with disease activity in patients with Takayasu's (TAK) and Giant cell arteritis (GCA) patients. MATERIALS AND METHODS: 23 Takayasu's arteritis, 9 Giant cell arteritis patients and 25 healthy volunteers were included in the study. Serum LIF levels were measured ELISA. RESULTS: The mean age of Giant cell arteritis patients was statistically significantly higher than the other groups (p<0.001). The rate of women was found to be higher in Takayasu's arteritis (p=0.021). When healthy control, patients with GCA and Takayasu arteritis were compared, there was a difference in LIF values (p=0.018). In subgroup analyzes, LIF values were found to be higher in GCA patients compared to healthy controls (p<0.05). There was no statistically significant correlation between LIF and CRP (Rho=-0.038, p=0.778), ESR (Rho=0.114, p=0.399) and ITAS (Rho=-0.357, p=0.094). While CRP was statistically significantly higher in patients with disease activity (p=0.003), there was no statistically significant difference between patients in terms of ESR and LIF values. While there was a statistically significant relationship between CRP (OR=1.19 [1.03-1.37], p=0.018) and disease activity in univariate analyses, no statistically significant variable was found in multivariable analyses. CONCLUSIONS: LIF values were significantly higher in patients with Giant cell arteritis compared to healthy controls.

Prevalence and pattern of myocardial involvement on cardiac magnetic resonance imaging in Takayasu arteritis and its relationship with disease activity.

BACKGROUND: Cardiac involvement in Takayasu arteritis (TA) is the major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) is an excellent modality for the assessment of myocardial involvement. Studies have shown myocardial involvement in 25%-27% of patients. OBJECTIVES: To evaluate the prevalence and pattern of myocardial involvement in TA on CMR. We also evaluated any correlation between CMR changes and disease activity score (ITAS 2010 and ITAS-A) assessed at the time of CMR. METHODS: Patients classified as Takayasu arteritis according to Sharma et al. criteria were enrolled in the study. Demographic, clinical, and laboratory data were documented in the predesigned proforma. CMR was performed on a dedicated cardiac 3Tesla MR machine. Disease activity was recorded by ITAS2010 and ITAS-A. RESULTS: A total of 37 TA patients were included. Mean (±SD) age was 29±11 years. Female to male ratio was 3:1. Five patients (14%) had myocardial involvement on CMR. Two (2/5) had myocarditis and three (3/5) patients had features of ischaemic myocardial fibrosis. CONCLUSION: The myocardium is affected in TA, however the prevalence of subclinical myocardial involvement in our study was less (8% vs. 25%-27%) compared to the previous studies. Myocardial involvement trends towards early age of onset, less disease duration, lack of classical risk factors, and more with disease activity.

Prevalence and pattern of myocardial involvement on cardiac magnetic resonance imaging in Takayasu arteritis and its relationship with disease activity / Prevalencia y patrón de afectación miocárdica en la resonancia magnética cardíaca en la arteritis de Takayasu y su relación con la actividad de la enfermedad

Background: Cardiac involvement in Takayasu arteritis (TA) is the major cause of morbidity and mortality. Cardiovascular magnetic resonance (CMR) is an excellent modality for the assessment of myocardial involvement. Studies have shown myocardial involvement in 25%–27% of patients. Objectives: To evaluate the prevalence and pattern of myocardial involvement in TA on CMR. We also evaluated any correlation between CMR changes and disease activity score (ITAS 2010 and ITAS-A) assessed at the time of CMR. Methods: Patients classified as Takayasu arteritis according to Sharma et al. criteria were enrolled in the study. Demographic, clinical, and laboratory data were documented in the predesigned proforma. CMR was performed on a dedicated cardiac 3Tesla MR machine. Disease activity was recorded by ITAS2010 and ITAS-A. Results: A total of 37 TA patients were included. Mean (±SD) age was 29±11 years. Female to male ratio was 3:1. Five patients (14%) had myocardial involvement on CMR. Two (2/5) had myocarditis and three (3/5) patients had features of ischaemic myocardial fibrosis. Conclusion: The myocardium is affected in TA, however the prevalence of subclinical myocardial involvement in our study was less (8% vs. 25%–27%) compared to the previous studies. Myocardial involvement trends towards early age of onset, less disease duration, lack of classical risk factors, and more with disease activity.(AU)

Antecedentes: La afectación cardíaca en la arteritis de Takayasu (AT) es la principal causa de morbmortalidad. La resonancia magnética cardiovascular (RMC) es una modalidad excelente para la evaluación de la afectación miocárdica. Los estudios han demostrado afectación del miocardio en el 25-27% de los pacientes. Objetivos: Evaluar la prevalencia y patrón de afectación miocárdica en AT en RMC. También se evaluó cualquier correlación entre los cambios de RMC y la puntuación de actividad de la enfermedad (ITAS 2010 e ITAS-A) evaluada en el momento de la RMC. Métodos: Pacientes clasificados como arteritis de Takayasu según los criterios de Sharma et al. se inscribieron en el estudio. Los datos demográficos, clínicos y de laboratorio se documentaron en el formulario prediseñado. La RMC se realizó en una máquina de RM cardíaca de 3 Tesla dedicada. La actividad de la enfermedad fue registrada por ITAS 2010 e ITAS-A. Resultados: Se incluyeron un total de 37 pacientes con AT. La edad media (±DE) fue de 29±11 años. La proporción de mujeres a hombres fue de 3:1. Cinco pacientes (14%) tenían afectación miocárdica en la RMC. Dos (2/5) tenían miocarditis y 3 (3/5) pacientes tenían características de fibrosis miocárdica isquémica. Conclusión: El miocardio es afectado en la AT, sin embargo, la prevalencia de afectación miocárdica subclínica en nuestro estudio fue menor (8% vs. 25-27%) en comparación con los estudios previos. La afectación miocárdica tiende hacia una edad de inicio temprana, menor duración de la enfermedad, falta de factores de riesgo clásicos y más con la actividad de la enfermedad.(AU)

Takayasu arteritis associated with focal and segmentary glomerulosclerosis

Takayasu arteritis (TA) is a large vessel vasculitis that affects young people, related to cardiovascular outcomes and chronic kidney disease. We present the case of a 20-year-old male with a diagnosis of TA, who developed chronic kidney disease, impaired renal blood flow was ruled out, renal biopsy was compatible with focal and segmental glomerulosclerosis of a collapsing variety, other possible aetiologies were excluded. The mechanisms that mediate this association have not been determined, immune-mediated mechanisms are proposed. According to our review, this is the second reported case of this association and the first with a collapsing variety.

La arteritis de Takayasu es una vasculitis de grandes vasos que afecta a personas jóvenes y se relaciona con desenlaces cardiovasculares y enfermedad renal crónica. Se presenta el caso de un paciente masculino de 20 arios, con diagnóstico de arteritis de Takayasu, que desarrolla enfermedad renal crónica. Se descartan alteraciones en el flujo sanguíneo renal, en tanto que la biopsia renal resulta compatible con glomeruloesclerosis focal y segmentaria de variedad colapsante. Se excluyeron otras posibles etiologías. No se han determinado los mecanismos que median en esta asociación; se proponen mecanismos inmunomediados. Según nuestra revisión, se trata del segundo caso reportado de esta asociación y el primero con variedad colapsante.

Case report: Takayasu arteritis in a newborn, 5-years follow-up

Takayasu Arteritis (TA) is classified as a large vessel vasculitis, it primarily affects aorta and principal branches. The clinical presentation in pediatric patients is odd and there a few literature about it. The case is about a feminine patient hospitalized after birth with diagnosis of patent ductus arterious (PDA), abnormal widening of the descending aorta, aneurysm of the aortic arch. It is kept under close surveillance for 5 years, with imaging improvement in treatment with methotrexate and immunoglobulin, however she presented relapses on two occasions where the caliber decreased in descending aorta and left iliac artery. We can conclude that Identifying AT findings is important for early diagnosis, medical management, and proper monitoring specifically in pediatric patients where little literature is available.

La arteritis de Takayasu (AT) se clasifica como una vasculitis de grandes vasos desarrollándose sobre todo en aorta y ramas principales. Su presentación en pacientes pediátricos es rara y existe muy poca información en la literatura. Se trata de una paciente femenina hospitalizada al nacimiento, a quien se diagnostica ductus arterioso persistente (DAP), dilatación de aorta descendente y aneurisma de arco aórtico. Se mantiene bajo vigilancia estrecha durante cinco arios, con mejoría imagenológica en tratamiento a base metrotexato e inmunoglobulina, sin embargo, presenta recaídas en dos ocasiones, donde se observa disminución del calibre de aorta descendente y arteria iliaca izquierda. Se puede concluir que el identificar los hallazgos de la AT es importante para realizar un diagnóstico temprano, manejo médico oportuno y vigilancia adecuada, en específico en pacientes pediátricos, de quienes se tiene poca información en la literatura.

Chronic angina secondary to Takayasu arteritis. Report of one case / Síndrome coronario crónico secundario a aortitis y enfermedad coronaria ostial bilateral. Informe de un caso

Chronic coronary syndromes are usually considered uncommon in young women, related to slower progression of atherosclerotic coronary artery disease, have atypical clinical presentations, and experience less diagnostic investigation. Non-atherosclerotic causes of coronary artery disease should be considered in young women experiencing angina. We report a 25-year-old woman who consulted for five months of moderate exertion angina. Physical examination revealed a right carotid bruit and asymmetrical upper extremity peripheral pulses. Initial work-up and imaging allowed to diagnose aortitis with bilateral coronary ostial stenosis secondary to Takayasu's arteritis. The patient experienced an apparent clinical response to initial medical therapy. However, follow-up evaluation revealed persistence of significant ischemia and requirement for myocardial revascularization. A percutaneous coronary intervention was performed.

Los síndromes coronaries crónicos son infrecuentes en mujeres jóvenes, quienes suelen presentar una lenta progresión de enfermedad coronaria aterosclerótica, tienen presentación clínica atípica y son menos sujetas a exploración diagnostica. Se deben considerar causas no ateroscleróticas de enfermedad coronaria en mujeres jóvenes con angina. Informamos una paciente de 25 años que consultó por cinco meses de angina con esfuerzos moderados. Al examen físico presentaba un soplo carotideo derecho y pulsos asimétricos de extremidades superiores. La exploración de laboratorio inicial y posterior evaluación multimodal permitió evidenciar la presencia de aortitis y estenosis de ambos ostium coronarios, concordante con el diagnóstico de una arteritis de Takayasu. Inició terapia medica con respuesta clínica aparentemente favorable. No obstante, la evaluación cardiológica no invasiva en el seguimiento permitió corroborar la persistencia de isquemia significativa y necesidad de revascularización miocárdica. Se realizó una intervención coronaria percutánea de ambos ostium, con una evolución favorable.

Arteritis de takayasu. alternativas terapéuticas y pronóstico a largo plazo / Takayasu arteritis Therapeutic alternatives and long term outcomes

Resumen Se analizaron los resultados del tratamiento quirúrgico y endovascular a 7.5 años, rango intercuartilo (RIC) entre 2.6 y 12.5 años de seguimiento en 34 pacientes con arteritis de Takayasu. Se reali zaron en total 5 cirugías cardíacas centrales y 53 procedimientos vasculares,18 cirugías de bypass (33.9%) y 35 angioplastías (66.1%). Entre los 18 procedimientos quirúrgicos realizados, 6 (33.3%) presentaron eventos, mientras que en los 35 con intervención percutánea hubo 16 eventos (45.7%). La supervivencia actuarial y otras complicaciones vasculares (método de Kaplan y Meier) a 1,3,5 y 10 años fue: 80% (IC 95% entre 74 y 89%), 68% (IC 95% entre 58 y 79%), 65% (IC 95% entre 54 y 76%) y 47% (IC 95% entre 41 y 62%).Tanto la revascularización endovascular como la quirúrgica fueron inicialmente exitosas. En el seguimiento del tratamiento endovascular hubo una alta tasa de eventos con necesidad de revascularización repetida a un mismo vaso en el 41% de los casos. La cirugía tuvo mayor mortalidad en pacientes con valvulopatía aórtica, de aorta ascendente y enfermedad coronaria y carotidea combinada. La arteritis de Takayasu requiere frecuentemente revascularización debido a reestenosis y lesiones de novo. En su evolución alejada, el procedimiento quirúrgico ofreció mejores resultados con menor reestenosis.

Abstract The aim of this study was to describe the long term prognosis of 34 patients with Takayasu arteritis and the results of surgi cal and endovascular treatment. A total of 5 central surgeries and 53 endovascular procedures were performed including 18 bypass surgeries (33.8%) and 35 angioplasties (66.2%). The median follow-up was 7.5 years, in terquartile range [IQR] 2.6-12.5. Among the 18 bypass surgeries 6 (33.3%) had events, while in the 35 patients with endovascular treatment there were 16 events (45.7%). The overall 1-, 3-, 5-, and 10-year death and arterial complication-free survival rates were 80% (95% CI between 74 and 89%), 68% (95% CI between 58 and 79%), 65% (95% CI between 54 and 76%) and 47% (95% CI between 41 and 62%). Both revascularization techniques were initially successful. In long term follow-up there was a high restenosis recurrence rate with endovascular treatment requiring repeated revascularization to the same vessel in 41% of the cases. Surgery had higher mor tality in patients with aortic and ascending aortic valve disease, combined coronary artery disease and carotid disease. In long term follow up Takayasu arteritis frequently requires revascularization and restenosis or new lesions are common. Surgical treatment had better results with less restenosis than angioplasty.

Resultados a largo plazo de tocilizumab para el tratamiento de arteritis de Takayasu en la práctica clínica / Long-term results of tocilizumab for the treatment of Takayasu arteritis in clinical practice

La arteritis de Takayasu (AT) es una enfermedad rara descrita como una vasculitis granulomatosa que afecta a las arterias elásticas de gran calibre, fundamentalmente la aorta y sus ramas principales. Como consecuencia de la inflamación de estos grandes vasos, puede dar lugar a la formación de aneurismas, estenosis u oclusión vascular. Afecta principalmente a mujeres jóvenes y los síntomas incluyen: síncope, mareo, disminución o ausencia de pulso, y alteración de la visión. El tratamiento convencional se basa principalmente en la utilización de corticoides e inmunosupresores, aunque recientemente se ha comenzado a considerar el uso de fármacos biológicos. Tocilizumab es un anticuerpo monoclonal dirigido contra IL-6 utilizado fuera de ficha técnica en algunos pacientes para tratar esta patología. Presentamos dos casos clínicos que describen el uso a largo plazo de tocilizumab en pacientes con AT refractaria al tratamiento convencional. En ambos casos se observó una excelente respuesta a tocilizumab, con remisión de la sintomatología y reducción de las dosis de corticoides asociados. No se registraron eventos adversos asociados al tratamiento. (AU)

Takayasu arteritis (TA) is a rare disease described as a granulomatous vasculitis that affects large-caliber elastic arteries, mainly the aorta and its main branches. As consequence of inflammation of these great vessels, TA can lead to the formation of aneurysms, stenosis or vascular occlusion. This pathology mainly affects young women and symptoms include: syncope, dizziness, decreased or absent pulse, and visual impairments. Conventional treatment is mainly based on the use of corticosteroids and immunosuppressants, although biological drugs has recently begun to be considered. Tocilizumab is a monoclonal antibody directed against IL-6 used off-label in some patients to treat this pathology. We present two clinical cases describing the long-term use of tocilizumab in patients with TA refractory to conventional treatment. In both cases, an excellent response to tocilizumab with remission of symptoms and a reduction in associated corticosteroid doses was observed. No adverse events associated with treatment were collected. (AU)

Desenlaces obstétricos en pacientes con arteritis de Takayasu: serie de casos / Pregnancy outcomes in patients with Takayasu's arteritis: Case series

RESUMEN Objetivo: La actividad y el riesgo de recaída de la arteritis de Takayasu son bajos durante la gestación. Hasta el 40% de las pacientes puede tener desenlaces obstétricos desfavorables, por lo que es importante conocer su comportamiento clínico. Describimos las características clínicas y el desenlace obstétrico de gestantes con arteritis de Takayasu atendidas en un hospital de alta complejidad. Materiales y métodos: Evaluación retrospectiva de historias clínicas de gestantes con arteritis de Takayasu atendidas en el Hospital Universitario San Vicente Fundación de Medellín, Colombia, entre 2011 y 2018. Resultados: Se incluyó en el estudio a 6 pacientes con mediana de edad al diagnóstico de 17,5 arios (RI 9,25), al parto de 24 arios (RI 8,25) y con una duración de la enfermedad de 5,5 arios (RI 10,5). Del total, 3 pacientes tenían compromiso aórtico extenso; al parto, 3 pacientes esta ban activas y requirieron inmunosupresores, 5 tenían hipertensión arterial, una desarrolló preeclampsia en el segundo trimestre, una tenía insuficiencia mitral y tricuspídea grave con disminución de la fracción de eyección del ventrículo izquierdo; 2 tenían aneurismas (arte ria subclavia izquierda y aorta ascendente). Ningún embarazo resultó en aborto ni parto pretérmino; hubo 2 óbitos fetales, uno por restricción del crecimiento intrauterino e insuficiencia placentaria, y otro de etiología desconocida; ambas pacientes con actividad de la enfermedad, afección aórtica extensa e hipertensión arterial. Fueron por cesárea 5 par tos por indicación materna; no hubo disección aórtica, rotura aneurismática ni hemorragia cerebral. Conclusión: Las pacientes con enfermedad activa y afección aórtica extensa presentaron resultados obstétricos desfavorables, lo que indica que el inadecuado control de la vasculitis genera mayores complicaciones materno-fetales.

ABSTRACT Objective: The activity and risk of relapse of Takayasu's arteritis are low during pregnancy. Up to 40% of patients may have unfavorable obstetric outcomes therefore it is important to know their clinical behavior. We describe the clinical characteristics and obstetric outcome of pregnant women with Takayasu arteritis treated in a hospital of high complexity. Materials and methods: A retrospective evaluation of medical records of pregnant patients with Takayasu's arteritis treated at Hospital Universitario San Vicente Fundación in Medellin, Colombia between 2011 and 2018. Results: Six patients with a median age at diagnosis 17.5 (RI 9.25) years, at delivery 24 (RI 8.25) years, disease duration 5.5 (RI 10.5) years. Three patients had extensive aortic involvement; at delivery, 3 patients were active and required immunosuppressants, 5 had high blood pres sure, one developed preeclampsia in the second trimester, one had severe mitral and tricus pid insufficiency with a decreased ejection fraction of the left ventricle; 2 had aneurysms (left subclavian artery and ascending aorta). No pregnancy resulted in abortion or preterm birth; there were 2 fetal deaths, one due to intrauterine growth restriction and placental insuffi ciency and another of unknown etiology; both patients with disease activity, extensive aortic condition, and arterial hypertension. Five deliveries were by cesarean section by maternal indication; there was no aortic dissection, aneurysmal rupture or cerebral hemorrhage. Conclusion: Patients with active disease and extensive aortic compromise presented unfa vorable obstetric results, suggesting that inadequate control of vasculitis leads to greater maternal-fetal complications.

Anestesia obstétrica en paciente con arteritis de Takayasu / Obstetric anesthesia in a patient with Takayasu arteritis

Introducción: La arteritis de Takayasu es una enfermedad inflamatoria que afecta la aorta y sus ramas y muestra predilección por la población asiática. Objetivo: Describir la conducción anestésica en una paciente obstétrica con arteritis de Takayasu. Presentación del caso: Paciente femenina, de 20 años de edad, con embarazo de 39 semanas, antecedentes de arteritis de Takayasu. A los seis meses de gestación aparecieron los primeros síntomas de la enfermedad. Se confirma diagnostico a través de AngioTAC de tronco supraaórtico y vasos del cuello, además de Doppler carotideo. Se observa trastornos vasculares oclusivos, estenosis concéntrica de ambas carótidas y de principales ramas del cayado aórtico. Finalmente, se confirmó el diagnóstico de arteritis de Takayasu tipo I. Al término de su embarazo, se decide cesárea electiva para evitar el trabajo de parto. Conclusiones: La anestesia obstétrica para pacientes con esta enfermedad es altamente complicada por su compleja conducción, observación e interpretación de la monitorización multiparamétrica e imagenológica. Durante la conducción anestésica es imprescindible el control estricto hemodinámico, evitando síndrome de bajo flujo sanguíneo y daño isquémico materno o fetal(AU) .

Introduction: Takayasu arteritis is an inflammatory disease that affects the aorta and its branches, and shows a predilection for the Asian population. Objective: To describe anesthetic management in an obstetric patient with Takayasu arteritis. Case presentation: Female patient, 20 years old, 39 weeks of pregnancy, a history of Takayasu arteritis. At six months of gestation, the first symptoms of the disease appeared. Diagnosis is confirmed through CT angiography of the supra-aortic trunk and neck vessels, in addition to carotid Doppler. Occlusive vascular disorders, as well as concentric stenosis of both carotids and the main branches of the aortic arch are observed. Finally, the diagnosis of type I Takayasu arteritis was confirmed. At the end of her pregnancy, an elective cesarean section was decided to avoid labor. Conclusions: Obstetric anesthesia for patients with this disease is highly complicated due to its complex management, observation and interpretation of multiparametric and imaging monitoring. During anesthetic conduction, strict hemodynamic control is essential, avoiding low blood flow syndrome and maternal or fetal ischemic damage(AU)

[Renovascular hypertension secondary to Takayasu arteritis]. / Hipertensión arterial renovascular secundaria a arteritis de Takayasu.

Secondary arterial hypertension (HT) is an increase in blood pressure due to a recognisable cause. It is estimated that the prevalence of people with HT is around 5%-15%. The search for secondary HT in all hypertensive patients is neither feasible nor cost-effective. However, there are a series of signs that force us to discount its diagnosis such as newly diagnosed hypertension before the age of 40, acute worsening of hypertension, resistant hypertension, the presence of extensive organ injury or clinical-analytical alterations, etc. It is important to diagnosis these patients since treatments can be curative, especially in young people. We present the case of a 46-year-old man with hypertensive emergency and subarachnoid haemorrhage due to aneurysmal rupture. The study of secondary causes was diagnostic for renal artery stenosis due to Takayasu arteritis in the scarring stage.

Takayasu's arteritis: Ischaemic retinal arterial occlusion as a possible initial presentation. / Arteritis de Takayasu: obstrucción arterial retiniana isquémica como posible presentación inicial.

Ocular manifestations are observed in 25% of patients with Takayasu's arteritis. Its signs and symptoms can be very variable. The case is presented of a 41-year-old woman with progressive vision loss in her right eye secondary to ischaemic retinal arterial occlusion. After a systematic study, a protein purified derivative (PPD) skin test compatible with tuberculosis was found to be the only alteration. After ruling out other causes, and based on the initial suspicion of tuberculous retinal vasculitis, treatment was started with antimicrobial agents and systemic corticosteroids, without any therapeutic response. Eighteen months later, the patient developed acute kidney failure, secondary to right renal artery stenosis. The CT-angiography revealed a thickening of the aortic arch and its branches, and Takayasús arteritis was finally diagnosed. Therefore, emphasis is made on the importance of the ophthalmologist in the diagnosis of Takayasús arteritis, in which its ophthalmological manifestations can be an early sign of the disease.

Aortitis: Hallazgos radiológicos en relación a sus diversas etiologías / Aortitis: Radiological Findings in Relation to its Various Etiologies

Resumen La inflamación de la aorta (aortitis) es una patología poco frecuente, con etiología infecciosa (pseudoaneurisma micótico, sífilis) y no infecciosa (arteritis, aortitis idiopática, espondilitis anquilosante, entre otras) de difícil diagnóstico clínico y variable pronóstico. Por esa razón, la utilización de diversos métodos por imágenes, tales como la tomografía computada multidetector (TCMD), la tomografía computada por emisión de positrones (PET-TC), la resonancia magnética (RM) y ultrasonido (US) facilitan la identificación, seguimiento y tratamiento de esa entidad. El siguiente trabajo tiene como objetivo realizar una revisión y actualización bibliográfica acerca de la aortitis y sus diversas etiologías, ejemplificando con casos de nuestra institución.

Abstract Aortic inflammation (aortitis) is a rare pathology, with infectious (fungal pseudoaneurysm, syphilis) and noninfectious etiology (arteritis, idiopathic aortitis, ankylosing spondylitis, among others), it has a difficult clinical diagnosis and a variable prognosis. The use of various imaging methods such as multidetector computed tomography (MDCT), magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT) and ultrasound (US) facilitate the identification, monitoring and treatment of this entity. The following paper aims to perform a literature review and update about aortitis and its various etiologies, exemplifying cases of our institution.

Arteritis de Takayasu / Takayasu arteritis

Introducción: La arteritis de Takayasu es una vasculitis sistémica considerada como una enfermedad rara. Su mecanismo etiopatogénico se basa en un proceso inflamatorio que afecta la arteria aorta y sus grandes ramas. La ausencia de pulso, la angiodinia, los trastornos hipertensivos y la presencia de nódulos en miembros inferiores se describen como sus principales manifestaciones clínicas. Objetivo: Dar a conocer las principales manifestaciones clínicas que permiten la sospecha diagnóstica de la arteritis de Takayasu en la atención primaria de salud. Caso clínico: Se presenta el caso de una paciente de 36 años de edad, la cual acude con manifestaciones clínicas que hace que se sospeche y finalmente se realice el diagnóstico de arteritis de Takayasu. Conclusiones: La sospecha clínica de la enfermedad se basa en una adecuada historia clínica, epidemiológica y hallazgos de laboratorio, los cuales son perfectamente detectables en la atención primaria de salud(AU)

Introduction: Takayasu arteritis is a systemic vasculitis considered a rare disease. Its etiopathogenic mechanism is based on an inflammatory process that affects the aorta and its large branches. The absence of pulse, the angiodynia, the hypertensive disorders and the presence of nodules in the lower limbs are described as their main clinical manifestations. Objective: To present the main clinical manifestations that allows the diagnostic suspicion of Takayasu arteritis in primary health care. Clinical case: 36-year-old female patient, which presents clinical manifestations that allow the diagnosis of Takayasu arteritis. Conclusions: The clinical suspicion of the disease is based on adequate clinical, epidemiological and laboratory findings, which are perfectly detectable in primary health care(AU)

Comparative study of 18F-FDG PET/CT and CT angiography in detection of large vessel vasculitis. / Estudio comparativo de la PET/TC con 18F-FDG y la angiografía por TC en la detección de la vasculitis de grandes vasos.

OBJECTIVE: Compare 18F-FDG PET/CT and CTangio in the diagnosis of extracraneal large vessel involvement in patients with suspicion of large vessel vasculitis (LVV). MATERIAL AND METHODS: A retrospective database reviewed 59 patients with clinical suspicion of LVV undergoing 18F-FDG PET/CT and CTangio. In 55 patients PET/TC and CTangio were done simultaneously in the same machine and in 4 patients with a scan interval of<1 month. PET/CT analyses included qualitatively and quantitative analysis (ratio SUVmax 18F-FDG vessel/SUVmax liver). CTangio was assessed for concentric mural thickening, contrast wall enhancement and structural vascular changes as potential complications of vasculitis. RESULTS: 18F-FDG PET/CT and CTangio show high specificity (97.2%) for LVV diagnosis, with an excellent sensitivity for 18F-FDG PET/CT (95.6%) and lower for CTangio (60.9%), which leads to a high negative predictive value for 18F-FDG PET/CT (97.2%) and a high false negative rate for CTangio (39.1%). A 70% concordance between 18F-FDG PET/CT and CTangio was obtained (Kappa index 0.70± 0.095 (P<.001). CONCLUSION: The results show the greater potential of 18F-FDG PET/CT for the detection and extension of LVV. Therefore, 18F-FDG PET/CT should be exploited to the maximum and consider as the first line imaging technique in the extracranial diagnosis of LVV and its possible association with polymyalgia rheumatica. The addition of CTangio could be more indicated in patients with Takayasu arteritis and in long-standing and/or severe vasculitis since it increases the accuracy in the detection of possible vascular complications.

Diagnóstico de Arteritis de Takayasu, un caso infrecuente / Diagnosis of Takayasu's Arteritis, an infrequent case

Introducción: la arteritis de Takayasu es considerada como una rara enfermedad que afecta fundamentalmente a mujeres jóvenes donde produce alteraciones en la aorta y sus principales ramas. Esta vasculitis tiene su sustento epidemiológico en la inflamación de las paredes de los vasos sanguíneos lo que determina la intensidad y severidad de las manifestaciones clínicas de la enfermedad. Objetivo: socializar las principales manifestaciones clínicas, elementos epidemiológicos y exámenes complementarios que permiten realizar el diagnóstico de la arteritis de Takayasu. Caso clínico: se presenta el caso de una paciente de 46 años de edad la cual es referida desde la atención primaria de salud, con manifestaciones clínicas y de laboratorio que permiten confirmar el diagnóstico de la arteritis de Takayasu. Conclusiones: Las manifestaciones generales, oftalmológicas y cardiovasculares fueron las más representativas en este caso. La identificación de la misma, unidos a los elementos epidemiológicos y los resultados de los exámenes complementarios constituyeron los pilares diagnósticos de la enfermedad. Los esteroides e inmunosupresores son los grupos farmacéuticos más utilizados en el tratamiento de la AT. Un elevado por ciento de pacientes requieren tratamiento quirúrgico(AU)

Introduction: Takayasu arteritis is considered a rare disease that affects mainly young women where it produces alterations in the aorta and its main branches. This vasculitis has its epidemiological sustenance in the inflammation of the walls of the blood vessels which determines the intensity and severity of the clinical manifestations of the disease. Objective: to socialize the main clinical manifestations, epidemiological elements and complementary tests that allow the diagnosis of Takayasu arteritis. Clinical case: the case of 46-year-old patient is presented, which is referred from primary health care, with clinical and laboratory manifestations that confirm the diagnosis of Takayasu's arteritis. Conclusions: The general, ophthalmological and cardiovascular manifestations were the most representative in this case. The identification of the same, together with the epidemiological elements and the results of the complementary tests constituted the diagnostic pillars of the disease. Steroids and immunosuppressants are the pharmaceutical groups most used in the treatment of TA. A high percentage of patients require surgical treatment(AU)

Afectación cardiovascular en una paciente con arteritis de Takayasu, a propósito de un caso / Cardiovascular involvement in a patient with Takayasu arteritis, in relation to a case

Introducción: la arteritis de Takayasu es una vasculitis sistémica que provoca alteraciones en distintos sistemas de órganos, las más características y peligrosas ocurren en el sistema cardiovascular. Objetivo: socializar las manifestaciones cardiovasculares que con mayor frecuencia se presentan en el curso de la arteritis de Takayasu. Caso clínico: se presenta el caso de una paciente femenina, de 46 años de edad, con manifestaciones clínicas que permiten llegar al diagnóstico de arteritis de Takayasu; dentro del cuadro clínico se presentan múltiples afectaciones cardiovasculares que ensombrecen el pronóstico de la paciente. Conclusiones: las manifestaciones cardiovasculares en la arteritis de Takayasu no solo forman parte de los criterios diagnósticos y de las manifestaciones clínicas de la enfermedad; sino que también forman parte de las complicaciones de la arteritis y su presencia empeora la evolución clínica de la enfermedad y complica el pronóstico del paciente(AU)

Introduction: Takayasu's arteritis is a systemic vasculitis that causes alterations in different organ systems, the most characteristic and dangerous occur in the cardiovascular system. Objective: to socialize the cardiovascular manifestations that most frequently occur in the course of Takayasu's arteritis. Clinical case: the case of a female patient, 46 years of age, with clinical manifestations that lead to the diagnosis of Takayasu arteritis; Within the clinical picture there are multiple cardiovascular affectations that overshadow the prognosis of the patient. Conclusions: the cardiovascular manifestations in Takayasu's arteritis are not only part of the diagnostic criteria and the clinical manifestations of the disease; they are also part of the complications of arteritis and their presence worsens the clinical evolution of the disease and complicates the patient's prognosis(AU)

Arteritis de Takayasu y formación inusual de aneurisma gigante de arteria pulmonar, reporte de un caso / Takayasu arteritis and giant aneurysm of pulmonary artery, Case report

RESUMEN La arteritis de Takayasu es una de las vasculitis de grandes vasos que afecta a la aorta y a sus ramas principales, llevando a estenosis arterial y, en ocasiones, a trombosis. También se observa la formación de aneurismas en la circulación general. No tiene un tratamiento específico. La terapia de elección son los corticoides, seguidos de otros inmunosupresores en casos de no lograrse un control adecuado del proceso inflamatorio. Se reporta el caso de un paciente con arteritis de Takayasu, con formación inusual de aneurisma gigante de la arteria pulmonar.

ABSTRACT Takayasu arteritis is a large vessel vasculitis that affects the aorta and its main branches, leadingto arterial stenosis, and occasionally, thrombosis. There is also aneurysm formation in the general circulation. There is no specific treatment. The therapy of choice is corti-costeroids, followed by other immunosuppressants in cases where adequate control of the inflammatory process is not achieved. A case is presented of a patient with Takayasu arteritis and the unusual presence of giant aneurysm of pulmonary artery.

Sustained remission after long-term biological therapy in patients with large vessel vasculitis: an analysis of ten cases.

OBJECTIVES: To describe the results obtained in clinical practice with the use of biological therapy (BT) in patients diagnosed with Takayasu arteritis (TA) and giant cell arteritis (GCA). METHODS: Retrospective single center study of TA/GCA patients who received BT (infliximab [IFX], etanercept [ETN] and tocilizumab [TCZ]). In TA, active disease was defined according to a previous National Institutes of Health study. In GCA, active disease was defined with a modified criteria and clinical manifestations secondary to temporal artery involvement or polymyalgia rheumatica symptoms. Clinical data and outcomes are reported using descriptive statistics. RESULTS: Five patients with TA and 5 with GCA were included. The main reason for starting BT was lack of response to prior therapy and/or ≥2 relapses during GC tapering. Five patients started IFX, four TCZ and 1 ETN. Remission was observed before 6 months in all cases. Only one patient had a relapse during long-term follow-up and the overall GC daily dose was reduced by 70%. Two AEs were considered attributable to IFX and one to TCZ. CONCLUSION: A favorable and sustained response to BT was observed in our patients with TA and GCA. Thus, BT might be considered as an alternative in patients with large vessel arteritis refractory to conventional treatment or with GC related comorbidities.

Uropatía obstructiva recurrente en arteritis de Takayasu / Recurrent upper urinary tract obstruction in takayasu arteritis

La arteritis de Takayasu (AT) es una enfermedad inflamatoria infrecuente de vasos grandes. A menudo, el daño crónico originado en las grandes arterias requiere de revascularización mediante prótesis vasculares. Excepcionalmente la implantación de las prótesis origina lesiones en otros órganos, como los uréteres, los cuales pueden obstruirse por compresión extrínseca por el tejido fibroso retroperitoneal originado como reacción a la presencia de la prótesis vascular.Se comunica el caso de una mujer que presentó hidronefrosis bilateral en forma asincrónica después de los 2 años de la instalación de una prótesis aorto-ilíaca con insuficiencia renal aguda, la cual pudo revertirse mediante. (AU)

Takayasu's arteritis is a rare inflammatory disease of large vessels. Often, chronic damage resulting in large arteries stenosis requires revascularization with vascular prostheses. Exceptionally, the presence of prostheses causes lesions in other organs, such as ureters, which are subject to extrinsic compression caused by retroperitoneal fibrous tissue originated as a reaction to the presence of vascular prostheses. We report the case of a woman who presented bilateral asynchronous hydronephrosis after 2 years of the installation of an aorto- iliac graft with acute renal failure, which could be reversed by releasing the ureter from the periprosthetic fibrous retroperitoneal tissue.(AU)