Reporte de pacientes con espondiloartritis y arteritis de Takayasu en Argentina: ¿asociación causal o casual? / Report of patients with spondyloarthritis and Takayasu's arteritis in Argentina: is it a causal or casual association?

Introducción: tanto las espondiloartritis (EspA) como la arteritis de Takayasu (TAK) son enfermedades infrecuentes y su asociación es aún más rara. Objetivos: presentar una serie de pacientes con diagnóstico concomitante de EspA o con rasgos de EspA y TAK en Argentina, y realizar una revisión de la literatura respecto de esta asociación. Materiales y métodos: se recopilaron las características demográficas, clínicas y terapéuticas de pacientes con diagnóstico concomitante de EspA o con algunos rasgos de EspA y TAK, de distintos centros de salud de la República Argentina. Resultados: se describen 7 pacientes, de los cuales 4 presentaban EspA, uno con compromiso axial (EspAax) juvenil, otro con artritis psoriásica (APs), otro con espondilitis anquilosante (EA) y el último con EspAax pura, y 3 de ellos tuvieron rasgos de EspA (enfermedad Crohn, psoriasis y oligoartritis asimétrica de grandes articulaciones). En la mayoría de los casos, los rasgos de EspA se presentaron con una mediana de 4 años antes de la TAK. Conclusiones: varios reportes y series de casos relatan la superposición entre estas dos enfermedades. Si bien las mismas podrían compartir cierta base genética común, todavía no contamos con evidencia sólida que permita estimar que esta asociación no es casual.

Introduction: both spondyloarthritis (SpA) and Takayasu arteritis (TAK) are rare diseases, and their association is even rarer. Objectives: to present a series of patients with a concomitant diagnosis of SpA or with features of SpA and TAK in Argentina and review the literature regarding this association. Materials and methods: the demographic, clinical and therapeutic characteristics of patients with a concomitant diagnosis of SpA or with some features of SpA and TAK were collected from different health centers in Argentina. Results: 7 patients are described, of which 4 had SpA, one with juvenile axial involvement (axSpA), another with psoriatic arthritis (PsA), another with ankylosing spondylitis (AS) and the last patient with pure axSpA and 3 of them had features of SpA (Crohn's disease, psoriasis and asymmetric oligoarthritis of large joints). In most cases, SpA features presented a median of 4 years before TAK. Conclusions: several case reports and case series reported overlap between these two diseases. Although they could share a certain common genetic basis, we still do not have solid evidence that allows us to estimate that this association is not coincidental.

Use of intravenous lidocaine in a patient with refractory chest pain secondary to Takayasu arteritis. Case report / Uso da lidocaína endovenosa em paciente com dor torácica refratária secundária à arterite de Takayasu. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: Takayasu's arteritis (TA) is a rare form of chronic inflammatory disease involving large vessels, with uncertain etiology, with chest pain as a common and challenging symptom, resulting from inflammation in the aortic root or arch, pulmonary artery or coronary arteries. The objective of this study was to describe the use of intravenous lidocaine to treat severe and refractory chest pain secondary to TA. CASE REPORT: A 33-year-old female patient diagnosed with TA, with severe chest pain that was difficult to manage, was admitted after consulting an emergency department. The pain was unresponsive to traditional treatment after a week of drug adjustments. As a therapeutic option, a Sympathetic Venous Blockade (SVB) with lidocaine was chosen, achieving a reduction in pain from 10 to 3 on the Visual Analog Scale. Infliximab was administered before discharge. The patient was re-evaluated at an outpatient appointment after 30 days. CONCLUSION: This strategy for the treatment of severe chest pain allowed for pain reduction and relief.

RESUMO JUSTIFICATIVA E OBJETIVOS: A arterite de Takayasu (AT) é uma forma rara de doença inflamatória crônica envolvendo grandes vasos, com etiologia incerta, tendo a dor torácica como um sintoma comum e desafiador, consequente à inflamação na raiz ou arco aórtico, artéria pulmonar ou coronárias. O objetivo deste estudo foi relatar a utilização da lidocaína por via endovenosa na abordagem da dor torácica intensa e refratária secundária à AT. RELATO DO CASO: Paciente do sexo feminino, 33 anos, com diagnóstico de AT, dor torácica intensa de difícil manejo, internada após consulta em serviço de emergência. Dor não responsiva ao tratamento tradicional após uma semana de ajustes em fármacos. Como opção terapêutica, foi escolhido o Bloqueio Simpático Venoso (BSV) com lidocaína, obtendo redução da dor de 10 para 3 na Escala Analógica Visual. Antes da alta hospitalar foi administrado infliximabe. Paciente foi reavaliada em consulta ambulatorial após 30 dias. CONCLUSÃO: Esta estratégia fora tratamento da dor torácica intensa permitiu redução e alívio da dor.

A rare case of Takayasu Arteritis in young woman with heart failure - A case report

Takayasu arteritis (TA) is a rare, systemic, granulomatous primary vasculitis of medium and large arteries. The name comes from Dr. Mikito Takayasu, who reported the problem in 1905 for the first time. It is also called as Pulseless Disease or Aortic Arch Syndrome and usually seen before 40 years with female and male ratio being 10:1. Takayasu arteritis is a major cause of high blood pressure levels in teenagers and young adults. Around 75 percent of the people having Takayasu get diagnosed usually at an average age of 29 years though they begin to show the symptoms at their teenage years because the early symptoms of Takayasu are nonspecific and common. Heart failure as the first presentation of the TA is rare but has been reported. Angiographic studies help in the diagnosis of Takayasu and patients usually respond to steroid therapy. We report a 16 years old female presented with history of upper limb claudication, dyspnea, orthopnea, non palpable pulse in bilateral upper limbs with non recordable BP, lower limb with high blood pressure recordings and bilateral carotid Bruit present. Imaging studies revealed circumferential wall thickening of arch of aorta, bilateral carotids, left sub clavian, left axillary. 2D echo revealed global hypokinesia with severe left ventricular dysfunction. Takayasu arteritis with heart failure diagnosis was made and administration of steroids, diuretics and ACE inhibitor improved the condition.

Takayasu's Arteritis with Aortic Insufficiency as Initial Presentation: A Case Report

Takayasu's disease is the first cause of inflammatory aortitis in young subjects. The disease preferentially affects the women. Takayasu's disease could be a mode of non-specific reaction of the aorta to various infectious or dysimmune etiological factors. Aortic insufficiency is present in 7 to 10% of cases. It is usually moderate. Its mechanism is not unambiguous: direct damage to the aortic valves or dilation of the aortic annulus. Coronary damage responsible for angina or even myocardial infarction are also possible. We report the case of a severe aortic insufficiency secondary to takayasu disease complicated by acute coronary syndrome in a 42-year-old woman.

A rare case of carotid stroke in young due to Takayasu Arteritis with positive anti- cardiolipin antibodies � A case report

Takayasu arteritis is a well-known yet rare form of large vessel vasculitis. Takayasu arteritis affects mainly women, and is most commonly seen in Japan, South East Asia, India, and Mexico, where it usually presents in the 2nd or 3rd decade of life. It is seen usually as pulseless disorder often with bruit at the stenosed arteries. Manifestations range from asymptomatic disease, to catastrophic strokes. Angiography remains the gold standard for diagnosis. Approximately half of those patients treated with steroids will respond, and half of the remaining patients respond to methotrexate; mycophenolatemofetil may be useful. Fertility is not adversely affected and pregnancy does not appear to exacerbate the disease, although management of hypertension is essential. We herewith report a rare case of an11 year old girl, who presented with left-sided hemiparesis, dysarthria, left UMN facial palsy, feeble pulses on right side, high Blood Pressure recordings and positive anti- cardiolipin antibodies. Imaging studies revealed Occluded right Common carotid artery, occluded right subclavian artery and stenotic right renal artery and MRI showed Acute Infarcts in Right Basal Ganglia and Right High Parietal Region, Hemorrhagic infarct in right MCA subcortical area.The diagnosis of Takayasu arteritis with recent cerebrovascular accident (left hemiparesis) with hypertension was made and the patient was started on steroids, anti-platelets, anti-hypertensives and physiotherapy.

Clinical characteristic of aortitis in cardiac surgery in a single center / 中国胸心血管外科临床杂志

@#Objective    To review and analyze the clinical manifestations of common aortitis in cardiac surgery. Methods    We screened 41 552 patients who were hospitalized in the Department of Cardiac Surgery of Zhongshan Hospital, Fudan University from 2010 to 2020, analyzed the patients' clinical data, and classified according to the type of diseases. Then we summarized all their clinical manifestations. Results    In our center 145 patients were operated for aortitis diseases, including 75 males and 70 females, with the age of 24-76 (45.6±11.3) years. There were 61 patients of Takayasu's arteritis, 51 patients of Behcet's disease, 8 patients of syphilitic aortitis, 8 patients of systemic lupus erythematosus, 2 patients of Kawasaki disease, 4 patients of ankylosing spondylitis, 10 patients of dry syndrome, and 1 patient of scleroderma. Conclusion    Aortitis is not uncommon in cardiac surgery, and awareness of the disease should be enhanced. So that we can distinguish various types of aortitis and to make proper management to improve patients' prognosis.

Analysis of differentially expressed microRNA and protein expression profiles carried by exosomes in the plasma of patients with Takayasu′s arteritis / 中华内科杂志

Objective:To detect the microRNAs (miRNAs) and proteins carried by exosomes in the plasma of patients with newly diagnosed Takayasu′s arteritis (TAK) and analyze their possible roles in the pathogenesis of TAK.Methods:Ten patients with newly diagnosed TAK from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital were selected during June-November 2020. Five healthy controls were matched with five patients by age and sex. RNA sequencing and protein mass spectrometry were used to detect miRNAs and proteins, respectively, carried by exosomes in the plasma. Differentially expressed miRNAs (DE-miRNAs) and proteins (DEPs) were screened. Thereafter, hierarchical cluster analysis, function, signal pathway, and protein domain enrichment analysis of DE-miRNAs and DEPs were performed. Finally, miRNAs and proteins related to vasculitis and autoimmunity were identified. The possible roles of the miRNAs and proteins in the pathogenesis of TAK were explored. Enumeration data were compared using Fisher′s exact probability test or Chi-square test, and a P-value<0.05 was considered significant. Results:Compared with the healthy controls, patients with TAK had 29 DE-miRNAs on their plasma exosomes. Among these DE-miRNAs, miR-101-3p, miR-122-5p, miR-143-3p, miR-185-3p, miR-192-5p, miR-194-5p, miR-19a-3p, miR-19b-3p, miR-20b-5p, miR-21-5p, miR-22-3p, miR-335-5p, miR-34a-5p, miR-3613-5p, miR-548ad-5p, miR-590-3p, and miR-7-5p were upregulated; whereas miR-1249-3p, miR-141-3p, miR-199a-5p, miR-199b-5p, miR-200a-3p, miR-200c-3p, miR-204-5p, miR-29c-5p, miR-335-3p, miR-381-3p, miR-4433b-5p, and miR-584-5p were downregulated. Finally, miR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, and miR-21-5p were identified. Among the 357 DEPs screened, 236 DEPs were upregulated, whereas 121 DEPs were downregulated. Finally, kallikrein B1 (KLKB1), kininogen 1 (KNG1), desmoplakin (DSP) were identified.Conclusion:MiR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, miR-21-5p, KLKB1, KNG1, and DSP carried by exosomes in plasma might participate in the pathogenesis of TAK by regulating vascular physiology, inflammation, autoimmunity, and other processes. They may be biomarkers and therapeutic targets of TAK.

Impaired myocardial structure and function assessment by cardiac MR in Takayasu arteritis with pulmonary artery involvement / 中华放射学杂志

Objective:To analyze the late gadolinium enhancement (LGE) manifestations, cardiac function, and myocardial strain by feature tracking (FT) in Takayasu arteritis (TA) with pulmonary artery involvement (PTA) using cardiac MR (CMR), and then to investigate manifestations of the impaired myocardial structure and function.Methods:A retrospective study was performed on 32 patients with PTA and 21 healthy subjects without cardiopulmonary diseases from January 2017 to December 2020. All of them underwent CMR examinations. According to the presence of pulmonary arterial hypertension (PAH),PTA patients were divided into two groups including PAH group (11 cases) and non-PAH group (21 cases). LGE manifestations were observed and Fisher exact test was used for statistical analysis between the two groups. Cardiac function parameters and FT values including global peak strain of the left and right ventricle were calculated separately in PAH, non-PAH group of patients and healthy controls, using One-way ANOVA or non-parametric Kruskal-Wallis test for statistical analysis including a pairwise comparison between groups. The correlations between FT values of the PAH group and parameters measured by right heart catheterization test (RHC) and transthoracic echocardiography were analyzed using Pearson or Spearman correlation analysis.Results:There were 23 PTA patients (71.9%) with LGE. LGE in the interventricular insertion points (IPs)(11/11), and in the mid-wall (11/11) or epicardial (10/11) myocardium was more common ( P values were 0.006,<0.001 and 0.011, respectively) in PAH group, compared with LGE in the IPs (11/21), and in the mid-wall (7/21) or epicardial (9/21) myocardium in non-PAH group. The absolute values of left ventricular global peak circumferential strain (LVGPCS), left ventricular global peak longitudinal strain (LVGPLS) and right ventricular global peak longitudinal strain in PAH group were smaller than those in healthy subjects ( P<0.05). The absolute values of LVGPCS and LVGPLS in non-PAH group were smaller than those in healthy subjects ( P<0.05). In PAH group, mean pulmonary artery pressure of RHC was correlated with several FT parameters ( P<0.05), especially left ventricular global peak radial strain ( r=-0.807, P=0.009) and LVGPCS ( r s=0.817, P=0.007). Conclusions:Myocardial injury can be seen in PTA patients. And LGE in the IPs and LGE in the mid-wall or epicardial myocardium is more common in PTA patients with PAH. LVGPCS and LVGPLS can early indicate left heart dysfunction in PTA patients without PAH.

Clinical characteristics of infantile Takayasu arteritis complicated with cardiac involvements / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of infantile Takayasu Arteritis (TAK) complicated with cardiac involvements.Methods:The clinical data and cardiac lesions of infantile TAK were collected retrospectively, and the clinical characteristics of the disease were analyzed and summarized. Mainly using decriptive statistical methods.Results:In these 20 cases, 16 cases (80%) had cardiac involvements, only 2 cases had related symptoms. The common lesions were coronary artery lesion (CAL), valvular disease, and elevated myocardial enzymes, while the rare lesions were arrhythmia, pericardial effusion, hypertensive heart disease, and heart failure. One case had acute heart failure, which was systolic heart failure and was accompanied by hypertensive heart disease. All 14 patients with CAL were found by conventional coronary ultrasound screening. A total of 39 CAL were found, all of which were coronary artery dilation, and the left main coronary artery was involved. Five patients had heart valve disease, all of them were valve insufficiency. The involved valves were mitral and tricuspid valves, and one of them was severe insufficiency. Arrhythmias were found in 2 cases, of which P1 was found to have paroxysmal atrial tachycardia with high atrioventricular block at 3 months. All 20 children survived and were in stable condition after being treat with biological agents and/or glucocorticoids. A case of hypertensive heart disease complicated with heart failure was followed up for 4 years, and the cardiac function and blood pressure returned to normal. Fourteen children with CAL lesions were given oral aspirin disease, the CALs disappeared in 10 cases and retracted in 4 cases. During the follow-up of 5 children with heart valves, insufficiency disappeared in 4 cases and improved in 1. No child underwent valve replacement during the follow-up. One of the children with arrhythmia was treated with antiarrhythmic drugs. After treatment, the arrhythmia disappeared. Now they have been followed up for 5 years without recurrence.Conclusion:Infantile TAK has a high incidence of heart involvement, with extensive lesions but insidious clinical symptoms. CALs are common, and heart failure is rare. It should be evaluated and treated as early as possible.

Successful Use of a Stent-Graft for Treating a Pseudoaneurysm Located at the Anastomosis of the Ascending Aorta and the Great Saphenous Vein / 日本心臓血管外科学会雑誌

Takayasu Arteritis causes annuloaortic ectasia and coronary ostial stenosis, which may necessitate open heart surgery. However, pseudoaneurysms are sometimes observed postoperatively, making subsequent treatment difficult. We report thoracic endovascular aortic repair of a pseudoaneurysm of the ascending aorta with an anastomosis of the great saphenous vein in a 61-year-old female with a history of multiple open-heart procedures. Thirty years earlier, she underwent aortic valve replacement and coronary artery bypass surgery for aortic regurgitation, and right coronary ostial stenosis. Eleven years after surgery, an ascending aortic aneurysm was found and Bentall's surgery was performed. Multiple open thoracotomies were subsequently performed. Postoperatively, a pseudoaneurysm was found at the anastomosis between the ascending aorta and the great saphenous vein. The patient was transferred to the emergency room owing to hemoptysis and was diagnosed with a ruptured pseudoaneurysm at the anastomosis of the ascending aorta and the great saphenous vein. By inserting a stent graft into the ascending aorta, we avoided further complications and her prognosis was good. She was discharged on postoperative day 18 and did not experience any end leak for a year. Thoracic endovascular aortic repair in the ascending aorta is a minimally invasive procedure that may be useful for high-risk patients.

Preliminary Results of Single-cell Transcriptome Sequencing in Renal Arterial Lesions of Takayasu Arteritis / 中国医学科学院学报

Objective To explore the preliminary application of single-cell RNA sequencing (scRNA-seq) in the renal arterial lesions in Takayasu arteritis (TA) patients. Methods This study included 2 TA patients with renal artery stenosis treated by bypass surgery in the Department of Vascular Surgery,Beijing Hospital.The obtained 2 renal artery samples were digested with two different protocols (GEXSCOPE kit and self-made digestion liquid) before scRNA-seq and bioinformatics analysis. Results A total of 2920 cells were obtained for further analysis.After unbiased cluster analysis,2 endothelial cell subsets,2 smooth muscle cell subsets,1 fibroblast subset,2 mononuclear macrophage subsets,1 T cell subset,and 1 undefined cell subset were identified.Among them,the two subsets of smooth muscle cells were contractile and secretory,respectively.The results of scRNA-seq indicated that enzymatic hydrolysis with GEXSCOPE kit produced a large number of endothelial cells (57.46%) and a small number of immune cells (13.21%).However,immune cells (34.64%) were dominant in the cells obtained by enzymatic hydrolysis with self-made digestive liquid. Conclusion scRNA-seq can be employed to explore the cellular heterogeneity of diseased vessels in TA patients.Different enzymatic digestion protocols may impact the proportion of different cells.

Evaluation of obstetric outcomes in Brazilian pregnant women with Takayasu arteritis

Abstract Objective Takayasu arteritis (TAK) is a rare chronic granulomatous vasculitis that affects large vessels and usually begins in women of childbearing age, so it is not uncommon for pregnancies to occur in these patients. However, there is limited information about these pregnancies, with reports of adverse maternal and obstetric outcomes. The objective of this study is to evaluate adverse maternal, fetal and neonatal events in pregnant patients with TA. Methods This is a cross-sectional study with retrospective data collection. We reviewed 22 pregnancies in 18 patients with TAK, according to the American College of Rheumatology criteria, that were followed up in a high-risk prenatal clinic specialized in systemic autoimmune diseases and thrombophilia (PrAT) at Hospital Universitário Pedro Ernesto, from 1998 to 2021. Results In twenty-two pregnancies, the mean age of patients was 28.09 years and the mean duration disease was 10.9 years. Of the 18 patients with TAK studied, only one had the diagnosis during pregnancy and had active disease. All other patients had a previous diagnosis of TAK and only 3 had disease activity during pregnancy. Twelve patients (66.6%) had previous systemic arterial hypertension and eleven (61.1%) had renal involvement. Among maternal complications, eight patients (36.3%) developed preeclampsia and six (27.2%) had uncontrolled blood pressure without proteinuria, while 10 (45%) had puerperal complications. Four (18.1%) births were premature, all due to severe preeclampsia and eight newborns (34.7%) were small for gestational age. When all maternal and fetal/neonatal outcomes included in this study were considered, only 6 (27.2%) pregnancies were uneventful. Conclusion Although there were no maternal deaths or pregnancy losses in this study, the number of adverse events was considerably high. Hypertensive disorders and small for gestational age newborns were more common than general population, while the number of patients with active disease was low. These findings suggest that pregnancies in patients with TAK still have several complications and a high-risk prenatal care and delivery are necessary for these patients.

Arteritis de Takayasu como hallazgo incidental en un paciente con enfermedad celiaca: la importancia de la tomografía computarizada por emisión de positrones / Takayasu's arteritis as an incidental finding in a patient with celiac disease: the importance of positron emission computed tomography

Resumen Introducción: La arteritis de Takayasu es una vasculitis de grandes vasos que afecta a las arterias elásticas de gran calibre, fundamentalmente la aorta y sus ramas principales. Ocurre frecuentemente en mujeres entre los 20-30 años, por lo que es muy infrecuente en la edad pediátrica. Caso clínico: Se presenta el caso de una paciente de 15 años en seguimiento desde los 9 años por enfermedad celiaca. A los 13 años se detectó anemia de trastornos crónicos, elevación de proteína C reactiva y velocidad de sedimentación globular. La paciente permaneció en todo momento asintomática. Tras descartar otros procesos, se solicitó tomografía computarizada por emisión de positrones (PET-TC), donde se detectaron lesiones compatibles con vasculitis de grandes vasos. La valoración por Cardiología evidenció un aneurisma en coronaria derecha. Se realizó angio-TC, que sugirió arteritis de Takayasu tipo III. Conclusiones: Es frecuente un retraso en el diagnóstico de la arteritis de Takayasu en los pacientes pediátricos. En este caso se encontraron lesiones de la fase II sin la presencia de síntomas correspondientes a la fase I. El PET-TC permitió el diagnóstico de vasculitis, clave para el diagnóstico de la paciente.

Abstract Background: Takayasu arteritis is a large-vessel vasculitis which affects large-caliber elastic arteries, primarily the aorta and its main branches. It mainly affects women between 20-30 years, so it is rare in children. Case report: We describe the case of a 15-year-old female who was followed up since she was 9 years old due to celiac disease. At the age of 13, anaemia of chronic disorders associated to elevated C-reactive protein and erythrocyte sedimentation were detected. The patient remained asymptomatic. After excluding other diseases, we requested a positron emission computed tomography (PET-CT); lesions compatible with large-vessel vasculitis were detected. Cardiology evaluation showed an aneurysm in the right coronary artery. Angio-CT suggested Takayasu type III arteritis. Conclusions: The delay in the diagnosis of Takayasu arteritis in pediatric patients is quite common. In this case, we have found phase II lesions, with no previous phase I symptoms. However, PET-CT allowed the diagnosis of vasculitis, key to the diagnosis of the patient.

Prevalence of subclinical systolic dysfunction in Takayasu's arteritis and its association with disease activity: a cross-sectional study

Abstract Background Takayasu's arteritis (TA) is a vasculitis that affects the aorta and its branches and causes stenosis, occlusion, and aneurysms. Up to 60% of TA patients are associated with cardiac involvement which confers a poor prognosis. Global longitudinal strain (GLS) analysis is an echocardiographic technique that can detect the presence of subclinical systolic dysfunction. Hence, this study aimed to describe the prevalence of subclinical systolic dysfunction in patients with TA using the GLS method and to correlate this finding with disease activity using the ITAS-2010 (Indian Takayasu Activity Score). Methods Thirty patients over 18 years of age who met the American College of Rheumatology (ACR) 1990 criteria for TA were included. The sample was submitted for medical record review, clinical and echocardiographic evaluation, and application of ITAS-2010. The cutoff for systolic dysfunction was GLS > - 20%. Results Of the 30 patients analyzed, 25 (83.3%) were female, and the mean age was 42.6 years (± 13.2). The median time since diagnosis was 7.5 years [range, 3-16.6 years], and the type V angiographic classification was the most prevalent (56.7%). Regarding echocardiographic findings, the median ejection fraction (EF) was 66% [61-71%] and the GLS was - 19.5% [-21.3 to -15.8%]. Although half of the participants had reduced GLS, only two had reduced EF. Eleven patients (33.%) met the criteria for activity. An association was found between disease activity and reduced GLS in eight patients (P = 0.02) using the chi-square test. Conclusion GLS seems to be an instrument capable of the early detection of systolic dysfunction in TA. The association between GLS and disease activity in this study should be confirmed in a study with a larger sample size.

Ascending Aortic Coarctation - an Atypical Location in a Non-Takayasu Arteritis Female Patient

ABSTRACT Coarctation of the aorta is a well-known congenital cardiovascular disorder that typically occurs within proximity to the ductus arteriosus. The ascending aorta, distal descending aorta, and abdominal aorta are segments which are prone to development of an atypical coarctation. The etiologies of atypical cases are usually associated with various types of vasculitis syndromes or underlying genetic disorders. In this report, we present a 24-year-old female patient with an ascending aortic coarctation which developed secondary to an atherosclerotic process.

Takayasu Arteritis as a Secondary Cause of Arterial Hypertension

Abstract Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.

Undiagnosed Takayasu Arteritis in Pregnancy

@#Takayasu arteritis is a chronic inflammation involving large vessels and it often occurs in young women of childbearing age. We described a case of a 29- year- old lady with previous history of proliferative ischemic retinopathy was noted to have low upper limbs blood pressure and weak upper limb pulses postpartumly. An urgent CT angiogram of thorax revealed features suggestive of large vessel vasculitis with involvement of ascending arch, descending aorta and its main branches, corresponding to type II TA . She was diagnosed to have Takayasu arteritis post delivery, and she underwent a successful pregnancy without intrapartum and postpartum complications. High index of suspicion must be given for pregnant patient who have persistent low blood pressure and weak pulse for early detection to avoid severe complications.

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.