[Aortitis]. / Aortites.

Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely, aortitis is histologically proven, as in some cases of clinically isolated aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain. Aortitis can be divided into different etiological categories: primary aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.

[Tocilizumab for refractory Takayasu arteritis with aortic aneurysm]. / Traitement par tocilizumab d'une artérite de Takayasu réfractaire avec anévrysme aortique.

Takayasu arteritis (TA) is a form of large vessel vasculitis (LVV) which affects the aorta and the main arteries. Many reports showed efficacy of biologic drugs (TNF α inhibitors and interleukin 6 inhibitors) in refractory TA cases. We report the case of a 46-year-old woman with refractory TA complicated by giant aortic aneurysm (AA) and severe hypertension, treated efficacy with tocilizumab (anti-interleukin 6 receptor monoclonal antibody).

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.

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features.

OBJECTIVE: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. METHODS: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit. RESULTS: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (±4.2) years and 1.2 (±1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. CONCLUSION: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

Leflunomide in Takayasu arteritis - A long term observational study.

OBJECTIVE: To evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. METHODS: An open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). RESULTS: The mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among TA patients who had changed leflunomide to another agent, two had an additional clinical relapse and needed to change therapy. CONCLUSION: Leflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

Difficulties in the differential diagnosis between Takayasu arteritis and rheumatic fever: case report.

In this article, we present the case of a patient with heart failure with biological aortic valve prosthesis and multiple vascular changes consistent with Takayasu arteritis (TA) who was seen in our department receiving corticosteroids and secondary prevention of rheumatic fever (RF); it was not possible to exclude the association between both diseases.

Association between rheumatic fever and Takayasu's arteritis - Case report.

Takayasu's arteritis (TA) and rheumatic fever are diseases that can start with cardiac features, making the diagnosis difficult. There are reports of association of RF with Takayasu's arteritis beginning with cardiac involvement in pediatric patients. The aim of this study is to report the possible association of RF and TA in patients with cardiac abnormalities. We describe the case of an adolescent initially diagnosed with RF who progressed with changes that allowed making the diagnosis of TA. TA and RF are two important causes of valve involvement that may have systemic manifestations.

[Pathogenesis of large vessel vasculitis]. / Physiopathologie des vascularites primitives des gros vaisseaux.

Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are two granulomatous vasculitis affecting large arteries that present specific epidemiological and clinical features. Their pathogenesis is not fully understood but major advances have been obtained during the last years, thus allowing the emergence of new therapeutic strategies. GCA and TA develop on a specific genetic background but share some similarities regarding the immunological pathways involved in their pathogenesis. The trigger of these diseases is not clearly identified but it is thought that an infectious agent could activate and lead to the maturation of dendritic cells that are localized in the adventitia of arteries. Then, the cells of the adaptative immune response are recruited and activated: CD4 T cells that polarize into Th1 and Th17 cells, cytotoxic CD8 T cells and Natural Killer cells. Furthermore, the T regulatory cells (Treg) are decreased both in GCA and TA. Humoral immune response seems also to be involved, especially in TA. Then, the cytokines produced by T lymphocytes (especially IL-17 and IFN-γ) trigger the recruitment and activation of monocytes and their differentiation into macrophages and multinuclear giant cells that produce IL-1ß and IL-6 that are responsible for general symptoms of GCA and TA, and cytotoxic mediators and growth factors that trigger the remodeling of the arterial wall leading to aneurysms and ischemic manifestations of GCA an TA.

Leflunomide in Takayasu arteritis - A long term observational study. / Leflunomida na arterite de Takayasu - Estudo observacional de longo prazo.

OBJECTIVE: To evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. METHODS: An open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). RESULTS: The mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among two TA patients who had changed laflunomide to another agent, two had a clinical relapse and needed to change therapy. CONCLUSION: Leflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

(18)F-Fluorodeoxyglucose positron emission tomography and serum cytokines and matrix metalloproteinases in the assessment of disease activity in Takayasu's arteritis.

OBJECTIVE: To evaluate (18)F-fluorodeoxyglucose ((18)F-FDG) uptake on positron emission tomography-computed tomography (PET-CT) and serum levels of different cytokines and matrix metalloproteinases (MMPs) in patients with Takayasu arteritis (TA) and associations with disease activity. METHODS: Serum levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-2, IL-6, IL-8, IL-12, IL-18, MMP-3 and MMP-9 were measured in 36 TA patients and 36 controls. Maximum standard uptake value (SUVmax) of (18)F-FDG in arterial walls was determined by PET-CT scans. TA patients were classified as active disease, inactive disease and possible active disease. RESULTS: Serum IL-6 and MMP-3 levels were higher in TA patients than in controls (p<0.001). Serum IL-6 was higher in patients with active disease and in patients with possible active disease than in inactive disease (p<0.0001). Patients with active disease had higher serum TNFα levels than patients with inactive disease (p=0.049) while patients with possible active disease presented higher IL-18 levels than patients with inactive disease (p=0.046). Patients with active disease had higher SUVmax values than those with inactive disease (p=0.042). By receiver operating characteristic (ROC) curve SUVmax was predictive of active disease in TA and values ≥1.3 were associated with disease activity (p=0.039). Serum TNF-α levels were higher in patients with SUVmax≥1.3 than <1.3 (p=0.045) and controls (p=0.012). Serum IL-6 levels were higher in patients with SUVmax≥1.3 than in controls (p<0.001). No differences regarding other biomarkers were found between TA patients and controls. CONCLUSIONS: Higher serum IL-6 and TNFα levels as well as higher (18)F-FDG uptake in arterial wall are associated with active TA.

[Chorea: a rare manifestation of Takayasu's arteritis]. / Coreia: uma manifestação rara da arterite de Takayasu.

The case of a girl with recurring chorea and a Takayasu's arteritis diagnosis is reported. This clinical manifestation has been reported in only one patient with this vasculitis in the pediatric group.

[18F-Fluorodeoxyglucose positron emission tomography and serum cytokines and matrix metalloproteinases in the assessment of disease activity in Takayasu's arteritis]. / Tomografia por emissão de pósitrons com 18F-fluordesoxiglicose e citocinas séricas e metaloproteinases da matriz na avaliação da atividade da doença na arterite de Takayasu.

OBJECTIVE: To evaluate 18F-fluorodeoxyglucose (FDG) uptake on positron emission tomography-computed tomography (PET-CT)-and serum levels of different cytokines and matrix metalloproteinases (MMPs) in patients with Takayasu's arteritis (TA) and associations with disease activity. METHODS: Serum levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-2, IL-6, IL-8, IL-12, IL-18, MMP-3 and MMP-9 were measured in 36 TA patients and 36 controls. Maximum standard uptake value (SUVmax) of 18F-FDG in arterial walls was determined by PET-CT scans. TA patients were classified as active disease, inactive disease and possible active disease. RESULTS: Serum IL-6 and MMP-3 levels were higher in TA patients than in controls (p<0.001). Serum IL-6 was higher in patients with active disease and in patients with possible active disease than in inactive disease (p<0.0001). Patients with active disease had higher serum TNFα levels than patients with inactive disease (p=0.049) while patients with possible active disease presented higher IL-18 levels than patients with inactive disease (p=0.046). Patients with active disease had higher SUVmax values than those with inactive disease (p=0.042). By ROC curve SUVmax was predictive of active disease in TA and values ≥1.3 were associated with disease activity (p=0.039). Serum TNF-α levels were higher in patients with SUVmax ≥1.3 than<1.3 (p=0.045) and controls (p=0.012). Serum IL-6 levels were higher in patients with SUVmax ≥1.3 than in controls (p<0.001). No differences regarding other biomarkers were found between TA patients and controls. CONCLUSIONS: Higher serum IL-6 and TNFα levels as well as higher arterial 18F-FDG uptake are associated with active TA.

Takayasu arteritis revisited.

Takayasu arteritis is a large vessel vasculitis with various clinical presentations depending on the territories affected. We report the case of a 47-year-old woman who was diagnosed with Takayasu arteritis following rapid progression of coronary disease. The condition evolved rapidly under corticosteroid therapy, with formation of new arterial stenoses within the carotid and splanchnic circulations. Disease remission was achieved with cyclophosphamide pulses and percutaneous angioplasty of the affected vessels was performed.

Hipertensão renovascular na arterite de Takayasu e angioplastia de artérias renais: resposta pressórica sustentada a longo prazo / Renovascular hypertension in Takayasu arteritis: long-term sustained pressoric response

A Arterite de Takayasu (AT) é uma vasculite de grandes vasos que acomete predominantemente pacientes jovens do sexo feminino. A estenose de artérias renais é frequente em pacientes hipertensos com AT. Uma paciente de 13 anos foi admitida em nosso serviço de Cardiologia com perda ponderal, febre, hipertensão arterial severa e insuficiência cardíaca descompensada em perfil B, com piora de função renal após introdução de inibidor da ECA. Apresentava assimetria de pulsos e de pressão arterial e sopros sobre artéria subclávia esquerda e artérias renais. O diagnóstico de Arterite de Takayasu foi dado. Arteriografia renal seletiva evidenciou estenose bilateral de artérias renais e a paciente foi submetida a angioplastia por balão com sucesso. Após o tratamento intervencionista, a paciente evoluiu clinicamente bem, com controle pressórico efetivo sustentado ao longo de 5 anos de seguimento. Em pacientes com AT e hipertensão renovascular, a angioplastia de artérias renais é factível e pode se traduzir em controle pressórico efetivo e benefícios clínicos a longo prazo

[Renal injury in Takayasu's arteritis]. / Atteinte rénale au cours de la maladie de Takayasu.

Renal involvement in Takayasu's arteritis is frequent and worsens the progression of the disease. This is primarily a renal artery stenosis causing renovascular hypertension. The glomerular disease is exceptional. This study was undertaken to determine the clinical, radiological, biological features and therapeutic response in patients with kidney disease associated with Takayasu arteritis. A retrospective chart review was conducted on 11 patients (five men and six females), with a mean age of 31.1 years (19-40 years). The discovery of kidney disease preceded the diagnosis of Takayasu's arteritis in eight cases. Ten patients developed hypertension. Laboratory finding showed proteinuria in five cases of which one case was due to nephrotic syndrome. Renal failure was found in six cases including four cases in stage of terminal chronic renal failure. Impairment of the renal artery was present in nine patients, proximal in seven cases and distal in two cases, bilateral in five cases and unilateral in four cases. Narrowing renal artery was found in seven cases. The renal biopsy revealed membranoproliferative glomerulonephritis in one case and nephrosclerosis in another case. Eleven patients were followed for an average period of 155 months (3-335 months). Remission of nephrotic syndrome was concomitant with the remission of the disease. Seven patients developed outbreaks of Takayasu's arteritis of which six were in care. Relapse of nephrotic syndrome was concomitant with the outbreak of the disease followed by spontaneous remission of both diseases. Improved pressure was obtained in 5 cases and worsening renal function in seven cases. Death was observed in two cases.

Takayasu arteritis: differential diagnosis in a teenager with severe acute kidney injury - a case report / Arterite de Takayasu: diagnóstico diferencial em adolescente com lesão renal aguda grave - relato de caso

ABSTRACT Takayasu arteritis (TA) is a chronic granulomatous inflammatory condition of unknown cause that involves large vessels - particularly the aorta and its branches - such as the carotid, coronary, pulmonary, and renal arteries. The left subclavian artery is the most frequently involved vessel. Stenosis of the renal artery has been reported in 23-31% of the cases and may result in malignant hypertension, ischemic renal disease, decompensated heart failure, and premature death. Involvement of both renal arteries is uncommon. Early onset anuria and acute kidney injury are rare and have been reported only in a few cases in the literature. This report describes the case of a 15-year-old female with constitutional symptoms evolving for a year, combined with headache, nausea, and vomiting, in addition to frequent visits to emergency services and insufficient clinical examination. The patient worsened significantly six months after the onset of symptoms and developed acute pulmonary edema, oliguria, acute kidney injury, and difficult-to-control hypertension, at which point she was admitted for intensive care and hemodialysis. Initial ultrasound examination showed she had normal kidneys and stenosis-free renal arteries. The patient was still anuric after 30 days of hospitalization. A biopsy was performed and revealed her kidneys were normal. Computed tomography angiography scans of the abdominal aorta presented evidence of occlusion of both renal arteries. The patient met the diagnostic criteria for Takayasu arteritis and had a severe complication rarely described in the literature: stenosis of the two renal arteries during the acute stage of ischemic renal disease.

RESUMO A Arterite de Takayasu (AT) é uma doença inflamatória crônica, granulomatosa, de causa desconhecida, que afeta grandes vasos, principalmente a aorta e seus ramos, incluindo artérias carótidas, coronárias, pulmonares e renais, sendo a artéria subclávia esquerda o vaso mais acometido. A estenose da artéria renal é relatada em 23-31% dos casos e pode resultar em hipertensão maligna, insuficiência renal por isquemia, descompensação cardíaca e morte prematura. O acometimento bilateral de artérias renais é incomum, sendo rara a presença de anúria súbita e lesão renal aguda como sintoma inicial da doença, com poucos relatos na literatura. O caso reporta uma adolescente de 15 anos com sintomas constitucionais durante um ano de evolução, associados a problemas como cefaleia, náuseas e vômitos, com idas frequentes a serviços de emergência, sem adequada investigação clínica. Após 6 meses do início dos sintomas, a paciente evoluiu de forma grave, com quadro de edema agudo de pulmão, oligúria, lesão renal aguda e hipertensão arterial de difícil controle, sendo necessário suporte em Unidade de Terapia Intensiva e hemodiálise. A ultrassonografia inicial mostrava rins normais e artérias renais sem sinais de estenose. Após 30 dias de internamento, paciente permanecia anúrica, sendo realizada biópsia renal que se mostrou dentro dos padrões da normalidade. Angiotomografia de aorta abdominal evidenciou oclusão bilateral de artérias renais. A paciente descrita fechou critérios diagnósticos para arterite de Takayasu e manifestou uma complicação grave pouco descrita na literatura: estenose bilateral de artérias renais, ainda na fase aguda da nefropatia isquêmica.

Primary angiosarcoma of the femur in a patient with Takayasu arteritis / Angiossarcoma primário do fêmur em um paciente com arterite de Takayasu

ABSTRACT Primary osseous angiosarcoma is a rare entity with variable biological behavior and poor prognosis. Little is known about the oncologic treatment and its etiology is still unknown. This study presents a case of lytic lesion in the right femur with dissemination to other bones, such as the vertebral column and skull, and to the lungs and central nervous system. Orthopedic surgery was performed in order to improve quality of life. Surgical specimen confirmed the diagnosis of high-grade malignant osseous angiosarcoma. Despite oncologic and orthopedic treatment, the patient had rapid and aggressive progression with a poor outcome.

RESUMO O angiossarcoma ósseo (AO) é uma patologia rara de comportamento biológico variável e com prognóstico reservado. Pouco se conhece sobre o seu tratamento oncológico e sua etiologia ainda é desconhecida. Os autores apresentam um caso de lesão lítica em fêmur proximal que se disseminou para outros ossos (tais como coluna e crânio), pulmão e sistema nervoso central. Foi instituído tratamento ortopédico, com vistas a uma melhoria da qualidade de vida e ao conforto do paciente. O diagnóstico de AO maligno de alto grau foi confirmado pelo espécime cirúrgico. Apesar disso e do tratamento oncológico feito, o paciente apresentou uma evolução rápida e agressiva com desfecho desfavorável.

Cerebral Aneurysm in Takayasu's Arteritis: A Case Report and Review of the Literature

Cerebral aneurysms are rarely observed in patients with Takaysu's arteritis. To our knowledge, this report presents the first case described in Brazil. Hemodynamic stress caused by obstructions of the cervical vessels developes cerebral aneurysms in these patients and they have higher rates of multiple and posterior circulation aneurysms than general population. In the majority of cases, microsurgical treatment is indicated mainly due to changes in the cervical vessels that preclude an endovascular approach.

Aneurismas cerebrais são raramente observados em pacientes com arterite de Takayasu. Este é o primeiro caso desta associação descrito no Brasil. O estresse hemodinâmico causado pelas obstruções vasculares cervicais desenvolve os aneurismas cerebrais nestes pacientes e eles apresentam maiores taxas de aneurismas múltiplos e de acometimento da circulação cerebral posterior. Na maioria dos casos, o tratamento microcirúrgico está indicado pois as alterações anatômicas dos vasos cervicais dificultam a abordagem endovascular.

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features / Estudo multicêntrico brasileiro de 71 pacientes com arterite de Takayasu juvenil: características clínicas e angiográficas

ABSTRACT Objective: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. Methods: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6 th to 12th month of diagnosis, and in the last visit. Results: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (± 4.2) years and 1.2 (± 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. Conclusion: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

RESUMO Objetivo: Descrever as características clínicas e angiográficas da arterite de Takayasu em crianças e adolescentes brasileiros. Métodos: Foi feita coleta retrospectiva de dados de 71 crianças e adolescentes acompanhados em 10 centros brasileiros de referência em reumatologia pediátrica. A avaliação foi feita em três tempos: início dos sintomas até o diagnóstico, do 6º ao 12º mês de diagnóstico e última consulta. Resultados: Dos 71 pacientes selecionados, 51 (71,8%) eram meninas. As médias de idade de início dos sintomas e de tempo até diagnóstico foram 9,2 anos (± 4,2) e 1,2 anos (± 1,4), respectivamente. No fim do estudo, 20 pacientes estavam em atividade de doença, 39 em remissão e cinco haviam evoluído a óbito. Os sintomas mais frequentes nas avaliação inicial, segunda avaliação e avaliação final foram, respectivamente, os constitucionais, os musculoesqueléticos e os neurológicos. A redução de pulsos periféricos foi o sinal cardiovascular mais frequente e a elevação da velocidade de hemossedimentação foi o achado laboratorial mais frequente nos três períodos de avaliação. O teste tuberculínico foi reagente em 41%. A estenose foi a lesão angiográfica mais encontrada, a artéria abdominal foi o segmento mais afetado e tipo angiográfico IV o mais frequente. A maioria (90%) fez terapia com glicocorticoides, 85,9% necessitaram de outro imunossupressor e 29,6% foram submetidos à angioplastia. Conclusão: Este é o maior estudo de arterite de Takayasu juvenil e nós observar elevado número de pacientes com idade inferior a 10 anos e a predominância de sintomas constitucionais no início da doença.