RESUMEN
OBJECTIVE: In this study, we aimed to investigate the role of erythrocyte sedimentation rate, C-reactive protein, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, monocyte/lymphocyte ratio, red blood cell distribution width, mean platelet volume, monocyte/HDL ratio, and C-reactive protein/albumin ratio in the diagnosis and treatment follow-up of active and remission Takayasu arteritis patients compared with healthy control group. METHODS: This is a retrospective case-control study in which 56 Takayasu arteritis patients and 40 age- and sex-matched healthy control were included. The blood values of Takayasu arteritis patients were analyzed during their active period and post-treatment remission periods, after comparing them with the healthy control. Furthermore, all parameters were evaluated by receiver operating characteristic analysis. RESULTS: Erythrocyte sedimentation rate, C-reactive protein, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, monocyte/lymphocyte ratio, monocyte/HDL ratio, and C-reactive protein/albumin ratio values were significantly higher in active Takayasu arteritis patients compared with healthy control and remission Takayasu arteritis groups. In the receiver operating characteristic analysis performed in active Takayasu arteritis and Takayasu arteritis patients in remission, C-reactive protein had the highest power to indicate disease activity, followed by C-reactive protein/albumin ratio, erythrocyte sedimentation rate, and monocyte/HDL ratio. When Takayasu arteritis in remission was compared with the healthy control, a significant difference was found between erythrocyte sedimentation rate, C-reactive protein, red blood cell distribution width, and C-reactive protein/albumin ratio, while no significant difference was found between monocyte/HDL ratio values. CONCLUSION: C-reactive protein/albumin ratio and red blood cell distribution width can be used in the diagnosis of Takayasu arteritis, and C-reactive protein/albumin ratio, red blood cell distribution width, and monocyte/HDL ratio measurements can be used in the follow-up. As C-reactive protein/albumin ratio is more powerful than C-reactive protein in differentiating the Takayasu arteritis group from the healthy control group, evaluation of C-reactive protein/albumin ratio together with albumin instead of evaluation of C-reactive protein alone when diagnosing the disease may help us to obtain more accurate results in daily practice.
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Sedimentación Sanguínea , Proteína C-Reactiva , Monocitos , Arteritis de Takayasu , Humanos , Arteritis de Takayasu/sangre , Arteritis de Takayasu/diagnóstico , Femenino , Proteína C-Reactiva/análisis , Estudios Retrospectivos , Adulto , Masculino , Estudios de Casos y Controles , Adulto Joven , Curva ROC , Estudios de Seguimiento , Biomarcadores/sangre , Persona de Mediana Edad , Albúmina Sérica/análisis , AdolescenteRESUMEN
Takayasu arteritis (TAK) is a granulomatous vasculitis that affects large arteries. T cells are important in TAK pathophysiology as these cells orchestrate granulomatous infiltration in arteries. This study aims to evaluate effector CD4+ T cells in the peripheral blood and the aortic wall of TAK patients and to analyze associations with disease activity and therapy. We performed a longitudinal study including 30 TAK patients and 30 controls. CD3+ T cells, CD3+CD4- T cells, CD4+ T cells, and Th1, Th2, and Th17 cells were evaluated in peripheral blood by flow cytometry, and the expression of CD4, CD8, Tbet, GATA-3, and RORγT was analyzed in the aorta of six patients by immunohistochemistry. TAK patients presented lower CD3+ T cells and CD4+ T cells (Pâ =â 0.031 and Pâ =â 0.039, respectively) than controls. Patients with active disease and those in remission had higher proportions of Th17 cells than controls (Pâ =â 0.016 and Pâ =â 0.004, respectively). Therapy for TAK did not result in significant differences concerning CD4+ effector T-cell subpopulations. Disease duration correlated with the number and percentage of Th2 cells (rhoâ =â -0.610 and rhoâ =â -0.463, respectively) and with Th17 cells (rhoâ =â -0.365 and rhoâ =â -0.568). In the aorta, the expression of CD8 was higher than CD4, whereas GATA-3, Tbet, and RORγT were expressed in this order of frequency. In conclusion, TAK patients present an increased Th17 response in the peripheral blood regardless of disease activity, whereas in the aortic tissue CD8 cells and the Th2 response were predominant.
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Aorta , Linfocitos T CD4-Positivos , Subgrupos de Linfocitos T , Arteritis de Takayasu , Humanos , Arteritis de Takayasu/inmunología , Arteritis de Takayasu/sangre , Femenino , Adulto , Masculino , Aorta/inmunología , Aorta/patología , Linfocitos T CD4-Positivos/inmunología , Subgrupos de Linfocitos T/inmunología , Miembro 3 del Grupo F de la Subfamilia 1 de Receptores Nucleares , Persona de Mediana Edad , Factor de Transcripción GATA3/metabolismo , Proteínas de Dominio T Box/metabolismo , Células Th17/inmunología , Adulto Joven , Estudios Longitudinales , Células Th2/inmunología , Células TH1/inmunologíaRESUMEN
INTRODUCTION: Takayasu's arteritis (TAK) patients are at an elevated risk of metabolic syndrome and cardiovascular diseases (CVD). Currently, there are no well-validated biomarkers to assess this risk in this population. Previous research in different cohorts has linked serum levels of osteoprotegerin (OPG) and its polymorphisms to accelerated atherosclerosis and a marker of poor prognosis in CVD. Thus, we assessed this protein as a potential biomarker of CVD in TAK patients. OBJECTIVES: To evaluate the serum levels of OPG and its SNPs (single nucleotide polymorphisms) in TAK patients and healthy controls, and to associate these parameters with clinical data. METHODS: This bicentric cross-sectional study included TAK patients who were compared with healthy individuals (control group). The serum levels of OPG and the frequency of OPG SNPs [1181G > C (rs2073618), 245 A > C (rs3134069), 163T > C (rs3102735), and 209 C > T (rs3134070)] were compared between the both groups and associated with clinical data. RESULTS: In total, 101 TAK patients and 93 controls were included in the study. The serum levels of OPG (3.8 ± 1.9 vs. 4.3 ± 1.8pmol/L, respectively; P = 0.059), and its four polymorphisms were comparable between both groups. In an additional analysis of only TAK patients, serum OPG levels and its four genes were not associated with any CVD parameters, except for higher OPG levels among patients without dyslipidemia. CONCLUSION: No significant differences were observed in serum OPG levels or in the genotype frequencies of OPG SNPs between the patient and control groups. Similarly, no correlation was found between laboratory parameters and clinical data on CVD risk in TAK patients.
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Biomarcadores , Osteoprotegerina , Polimorfismo de Nucleótido Simple , Arteritis de Takayasu , Humanos , Arteritis de Takayasu/genética , Arteritis de Takayasu/sangre , Osteoprotegerina/sangre , Osteoprotegerina/genética , Estudios Transversales , Femenino , Masculino , Adulto , Estudios de Casos y Controles , Biomarcadores/sangre , Persona de Mediana EdadRESUMEN
OBJECTIVE: This study aims to evaluate the effectiveness and safety of adalimumab (ADA) compared with leflunomide (LEF) in patients with Takayasu arteritis (TAK). METHOD: A retrospective cohort study was performed with the following inclusion criteria: the fulfilment of the 2022 American College Classification/European Alliance of Associations for Rheumatology criteria for TAK, age ≥18 years, and written informed consent. Forty-four patients were treated with LEF (n=28) or ADA (n=16) therapy due to relapsing/refractory disease or toxicity from previous therapy. Patients were evaluated at baseline (T0), at a median of 7.0 months (T1) and at 15.0 months of follow-up (T2). Data regarding disease activity, daily dose of prednisone, side effects and angiographic progression were analysed. RESULTS: LEF and ADA groups had similar features on the baseline visit. However, intravenous methylprednisolone was more frequently prescribed for the ADA group (p=0.019). On T1 and T2 visits, complete response rates were similar for ADA and LEF groups (75.0% and 88.5%; p=0.397 and 62.5% vs 78.3%; p=0.307), respectively. The differences remained non-significant after adjusting for baseline variables by propensity score matching. Although the ADA group had a higher median daily prednisone on visit T1 (p=0.004), it was similar on visit T2 (p=0.595). Similar rates of angiographic progression were observed in ADA and LEF groups (40% vs 25%; p=0.467). Mild-to-moderate adverse events were observed only in the LEF group (17.9%). CONCLUSION: LEF and ADA had comparable outcomes after a median of 15.0 months of follow-up. However, withdrawal from therapy and mild-to-moderate adverse events were only observed in the LEF group.
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Arteritis de Takayasu , Humanos , Adolescente , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/tratamiento farmacológico , Adalimumab/efectos adversos , Leflunamida/efectos adversos , Prednisona , Estudios RetrospectivosRESUMEN
Takayasu arteritis (TA) is a large-vessel vasculitis that rarely presents in infancy. Casitas B-lineage lymphoma (CBL) syndrome is a rare genetic disorder due to heterozygous CBL gene germline pathogenic variants that is characterized by a predisposition to develop juvenile myelomonocytic leukemia (JMML). Vasculitis, including TA, has been reported in several patients. Herein, we describe a patient with CBL syndrome, JMML, and TA, developing long-term remission of this vasculitis after allogeneic hematopoietic stem cell transplant (HSCT), and perform a literature review of CBL syndrome with vasculitis or vasculopathy. We report a female patient with growth delay, developmental issues, and congenital heart disease who was admitted at 14 months of age with massive splenomegaly, lymphadenopathy, fever, and hypertension. Body imaging studies revealed arterial stenosis and wall inflammation of the aorta and multiple thoracic and abdominal branches. Whole exome sequencing revealed a pathogenic variant in CBL with loss of heterozygosity in blood cells, diagnosing CBL syndrome, complicated by JMML and TA. Allogeneic HSCT induced remission of JMML and TA, permitting discontinuation of immunosuppression after 12 months. Six years later, her TA is in complete remission off therapy. A literature review identified 18 additional cases of CBL syndrome with vasculitis or vasculopathy. The pathogenesis of vasculitis in CBL syndrome appears to involve dysregulated T cell function and possibly increased angiogenesis. This case advances the understanding of vascular involvement in CBL syndrome and of the genetic, immune, and vascular interplay in TA, offering insights for treating CBL syndrome and broader TA.
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Trasplante de Células Madre Hematopoyéticas , Leucemia Mielomonocítica Juvenil , Arteritis de Takayasu , Humanos , Femenino , Arteritis de Takayasu/complicaciones , Leucemia Mielomonocítica Juvenil/diagnóstico , Leucemia Mielomonocítica Juvenil/genética , Leucemia Mielomonocítica Juvenil/terapia , Trasplante de Células Madre Hematopoyéticas/métodos , Mutación de Línea Germinal , Células GerminativasRESUMEN
BACKGROUND: Various studies have suggested interleukin 6 (IL-6) as a potential biomarker for detecting disease activity in Takayasu arteritis. METHODS: A systematic review and meta-analysis was performed to assess differences in IL-6 levels in patients with active (aTA) and inactive Takayasu arteritis (iTA), as well as healthy controls (HCs), using validated activity scores. Study quality and the risk of bias were assessed using STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) and the Newcastle-Ottawa and Joanna Briggs checklist, respectively. For the meta-analysis, we pooled the raw mean IL-6 levels in each group and then estimated and pooled the crude mean differences between the groups. We applied a random-effects model in all analyses. RESULTS: Of the 93 eligible articles, 10 were included after removing duplicates and studies that met the exclusion criteria. Overall, 1825 patients with a mean age ranging from 24 to 40.6 years were included. The pooled levels of IL-6 increased depending on disease activity as follows: HCs: 3.08 (95% confidence interval [CI], 0.88-5.28), iTA: 7.21 (3.61-10.82), and aTA: 22.67 (12.44-32.91) pg/mL. Patients with aTA had higher IL-6 levels than HCs (21.52 [95% CI, -0.43 to 43.47]) and those with iTA (16.69 [95% CI, 5.32-28.06]), whereas IL-6 levels were not different between HCs and patients with iTA (3.62 [95% CI, -13.18 to 20.42]). CONCLUSIONS: Interleukin 6 levels are significantly increased in patients with aTA compared with those with iTA and HCs but not in patients with iTA compared with HCs. More studies are needed to establish the IL-6 cutoff value for assessing disease activity.
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Interleucina-6 , Arteritis de Takayasu , Humanos , Adulto Joven , Adulto , Arteritis de Takayasu/diagnóstico , BiomarcadoresRESUMEN
Takayasu arteritis (TAK) is a rare systemic vasculitis primarily affecting the aorta and its major branches. Early diagnosis is critical to prevent severe vascular complications, yet current biomarkers are insufficient. This proof-of-concept study explores the potential of long non-coding RNAs (lncRNAs) in TAK, an area largely unexplored. In this cross-sectional study, 53 TAK patients, 53 healthy controls, and 10 rheumatoid arthritis (RA) patients were enrolled. Clinical evaluations, disease activity assessments, and lncRNA expression levels were analyzed. TAK patients exhibited significant dysregulation in several lncRNAs, including THRIL (19.4, 11.1-48.8 vs. 62.5, 48.6-91.4 arbitrary units [a.u.]; p < 0.0001), HIF1A-AS1 (4.5, 1.8-16.6 vs. 26.5, 19.8-33.7 a.u.; p < 0.0001), MALAT-1 (26.9, 13.8-52.5 vs. 92.1, 58.5-92.1 a.u.; p < 0.0001), and HOTAIR (8.0, 2.5-24.5 vs. 36.0, 30.0-43.8 a.u.; p < 0.0001), compared to healthy controls. Notably, HOTAIR (area under the ROC curve [AUC] = 0.825), HIF1A-AS1 (AUC = 0.820), and THRIL (AUC = 0.781) demonstrated high diagnostic potential with superior specificity (approximately 95%). While lncRNAs showed diagnostic promise, no significant correlations with TAK activity were observed. Comparative analysis with RA patients revealed distinct lncRNA expression patterns. This study unveils significant dysregulation of lncRNAs THRIL, HIF1A-AS1, and HOTAIR in TAK patients, underscoring their potential as biomarkers and opening avenues for further research into the mechanistic roles of these lncRNAs in TAK pathogenesis.
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Artritis Reumatoide , ARN Largo no Codificante , Arteritis de Takayasu , Humanos , ARN Largo no Codificante/genética , ARN Largo no Codificante/metabolismo , Arteritis de Takayasu/genética , Estudios Transversales , BiomarcadoresRESUMEN
An 18-year-old man with past medical history of secondary arterial hypertension diagnosed at age 15 presented with an abdominal magnetic resonance imaging (MRI) that exhibited left renal artery stenosis.
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Obstrucción de la Arteria Renal , Infarto del Miocardio con Elevación del ST , Arteritis de Takayasu , Masculino , Humanos , Adolescente , Infarto del Miocardio con Elevación del ST/diagnóstico , Infarto del Miocardio con Elevación del ST/etiología , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Constricción Patológica , Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/diagnóstico , Obstrucción de la Arteria Renal/etiologíaRESUMEN
OBJECTIVE: To develop the first evidence-based Pan American League of Associations for Rheumatology (PANLAR) guidelines for the treatment of Takayasu arteritis (TAK). METHODS: A panel of vasculitis experts developed a series of clinically meaningful questions addressing the treatment of TAK patients in the PICO (population/intervention/comparator/outcome) format. A systematic literature review was performed by a team of methodologists. The evidence quality was assessed according to the GRADE (Grading of Recommendations/Assessment/Development/Evaluation) methodology. The panel of vasculitis experts voted each PICO question and made recommendations, which required ≥70% agreement among the voting members. RESULTS: Eleven recommendations were developed. Oral glucocorticoids are conditionally recommended for newly diagnosed and relapsing TAK patients. The addition of nontargeted synthetic immunosuppressants (e.g., methotrexate, leflunomide, azathioprine, or mycophenolate mofetil) is recommended for patients with newly diagnosed or relapsing disease that is not organ- or life-threatening. For organ- or life-threatening disease, we conditionally recommend tumor necrosis factor inhibitors (e.g., infliximab or adalimumab) or tocilizumab with consideration for short courses of cyclophosphamide as an alternative in case of restricted access to biologics. For patients relapsing despite nontargeted synthetic immunosuppressants, we conditionally recommend to switch from one nontargeted synthetic immunosuppressant to another or to add tumor necrosis factor inhibitors or tocilizumab. We conditionally recommend low-dose aspirin for patients with involvement of cranial or coronary arteries to prevent ischemic complications. We strongly recommend performing surgical vascular interventions during periods of remission whenever possible. CONCLUSION: The first PANLAR treatment guidelines for TAK provide evidence-based guidance for the treatment of TAK patients in Latin American countries.
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Reumatología , Arteritis de Takayasu , Humanos , Estados Unidos , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/tratamiento farmacológico , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico , Inmunosupresores/uso terapéutico , Metotrexato/uso terapéuticoRESUMEN
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.
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Arteritis de Takayasu , Humanos , Femenino , Adolescente , Adulto , Masculino , Arteritis de Takayasu/diagnóstico por imagen , Arteritis de Takayasu/epidemiología , Estudios Transversales , Prevalencia , Constricción Patológica , EcocardiografíaRESUMEN
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.
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Coartación Aórtica , Enfermedades Cardiovasculares , Conducto Arterioso Permeable , Arteritis de Takayasu , Humanos , Femenino , Adulto Joven , Adulto , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico por imagen , Aorta Abdominal , Aorta TorácicaRESUMEN
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.
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Hipertensión , Preeclampsia , Complicaciones Cardiovasculares del Embarazo , Arteritis de Takayasu , Embarazo , Humanos , Femenino , Recién Nacido , Adulto , Resultado del Embarazo/epidemiología , Preeclampsia/epidemiología , Preeclampsia/etiología , Mujeres Embarazadas , Estudios Retrospectivos , Arteritis de Takayasu/diagnóstico , Brasil/epidemiología , Estudios Transversales , Complicaciones Cardiovasculares del Embarazo/epidemiología , Complicaciones Cardiovasculares del Embarazo/etiologíaRESUMEN
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.
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.
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Enfermedad Celíaca , Arteritis de Takayasu , Humanos , Femenino , Niño , Adolescente , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Electrones , Hallazgos IncidentalesRESUMEN
Takayasu arteritis is a chronic granulomatous vasculitis of unknown aetiology affecting the aorta and its major branches. Critical limb ischaemia may occur and eventually require surgical intervention. Surgical outcomes are influenced by disease activity, age, and comorbidities. We report a 43-year-old woman with Takayasu arteritis and stenosis of the left common iliac artery and occlusion of the left external iliac artery with limiting vascular claudication, who underwent angioplasty of the iliac artery with drug-eluting stent while being treated with infliximab. The artery ruptured a week later but was contained by the ilio-psoas muscle. She required subsequent stent placement to correct the lesion. Treatment comprised aspirin and clopidogrel, and the biological was switched to monthly intravenous tocilizumab. During an 8-year follow-up, serial imaging examinations showed a patent aorto-biiliac endoprosthesis, without evidence of thrombosis or restenosis. Clinically, the patient denies vascular claudication and pulses remain palpable in the left lower limb. This case highlights the risks inherent to these procedures in patients with large artery vasculitis and reinforces that the effectiveness of endovascular intervention can be increased by detailed preoperative evaluation, associated with a drug strategy including immunomodulatory and antiplatelet therapy as directed by the multispecialty team. Periodic imaging examinations are required because of the reported high rate of restenosis.
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Stents Liberadores de Fármacos , Arteritis de Takayasu , Femenino , Humanos , Adulto , Inhibidores de Agregación Plaquetaria/uso terapéutico , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/cirugía , Angioplastia , Isquemia/diagnóstico , Isquemia/etiología , Isquemia/terapia , Prótesis e ImplantesRESUMEN
BACKGROUND: Takayasu arteritis (TA) is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Diagnosis is usually delayed in most patients owing to the absence of typical clinical manifestations in the acute phase until the development of substantial arterial symptoms. The purpose of this case report is to describe abdominal pain being an uncommon symptom in TA. LEARNING OBJECTIVE: The clinical presentation of TA can be non-specific and can have serious consequences if the diagnosis is delayed, helping the clinicians who can treat with such patients (emergency medicine, cardiologist, or medical personnel).
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Arteritis de Takayasu , Humanos , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/tratamiento farmacológico , Aorta , Inflamación/complicaciones , Dolor Abdominal/diagnóstico , Dolor Abdominal/etiologíaRESUMEN
OBJECTIVE: Few population-based studies for Takayasu arteritis (TAK) have been performed, and Latin America prevalence/incidence data are unavailable. We aimed to understand TAK epidemiology in Rio de Janeiro City in 2020 (i.e., 6,747,815 inhabitants). METHODS: This was a cross-sectional fieldwork study where physicians who regularly followed TAK patients in public or private practices from Rio de Janeiro were invited to complete a REDCap survey. Patients should fulfill internationally accepted criteria for TAK and be living in the city. The 2020 prevalence was calculated as cases per 1,000,000 inhabitants (10 6 ). National government databases were analyzed for comparative prevalence assessment. The incidence rate was estimated using retrospective sections of cases diagnosed between 2010 and 2019; relative incidence risk was assessed by Poisson regression models with robust variance. RESULTS: Between May 2020 and May 2021, 114 patients were analyzed. Ninety-seven (85.1%) were female, and the most frequent races were White (44.7%), Mestizo (33.3%), and Black (16.7%). Takayasu arteritis 2020 prevalence was 16.9 cases/10 6 (95% confidence interval [CI], 14.1-20.3 cases/10 6 ); female patients and Black Brazilians had higher prevalence rates at 27.0 (95% CI, 22.2-33.3) and 25.1 cases/10 6 (95% CI, 16.1-39.3 cases/10 6 ), respectively. Government databases' analyses generated a lower prevalence (7.26 cases/10 6 ; 95% CI, 5.49-9.60 cases/10 6 ). The 2010-2019 mean incidence rate was 0.94 cases/10 6 per year (95% CI, 0.73-1.21 cases/10 6 ). Female patients had a higher risk than male patients of having TAK between 2010 and 2019 (relative risk, 2.70; 95% CI, 1.59-4.55; p < 0.0001). CONCLUSION: In the largest population-based fieldwork to date and the first Latin American study on TAK prevalence, Rio de Janeiro City in 2020 showed an intermediate prevalence between Europe and Asia. Female patients and Black Brazilians were more affected than the general population.
Asunto(s)
Arteritis de Takayasu , Humanos , Masculino , Femenino , Estudios Retrospectivos , Brasil/epidemiología , Arteritis de Takayasu/diagnóstico , Arteritis de Takayasu/epidemiología , Estudios Transversales , IncidenciaRESUMEN
The pathogenesis of Takayasu arteritis (TAK) is poorly understood and no previous studies have analyzed monocytes in TAK. This study evaluated monocyte subsets and monocyte-related chemokines in the peripheral blood of TAK patients and healthy controls (HC). Monocyte subsets were identified as classical (CD14+CD16-), intermediate (CD14+CD16dim), and non-classical (CD14dimCD16high) in the peripheral blood. The chemokines CCL (C-C chemokine ligand)2, CCL3, CCL4, CCL5, CCL7, CXCL (C-X-C motif ligand)10, and CX3CL (C-X3-C motif ligand)1 were measured in the sera. Thirty-two TAK patients and 30 HC were evaluated. Intermediate monocytes were higher in TAK than HC [25.0 cells ×106/L (16.7-52.0) vs. 17.2 cells ×106/L (9.2-25.3); p = 0.014]. Active disease was associated with monocytosis (p = 0.004), increased classical (p = 0.003), and intermediate (p < 0.001) subsets than HC. Prednisone reduced the percentage of non-classical monocytes (p = 0.011). TAK patients had lower CCL3 (p = 0.033) and CCL4 (p = 0.023) levels than HC, whereas CCL22 levels were higher in active TAK compared to the remission state (p = 0.008). Glucocorticoids were associated with lower CXCL10 levels (p = 0.012). In TAK, CCL4 correlated with total (Rho = 0.489; p = 0.005), classical and intermediate monocytes (Rho = 0.448; p = 0.010 and Rho = 0.412; p = 0.019). In conclusion, TAK is associated with altered counts of monocyte subsets in the peripheral blood compared to HC and CCL22 is the chemokine with the strongest association with active disease in TAK.