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

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

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

Rol de las imágenes en la patología arterial no ateroesclerótica poco frecuente en el abdomen / Role of images in rare vascular pathology in the abdomen

Resumen La patología arterial no ateromatosa constituye un grupo de patologías poco frecuentes, pero de gran importancia debido a su morbilidad y mortalidad asociadas. La presentación clínica de estas entidades es inespecífica, por lo que el estudio inicial con imágenes es fundamental. Debido a esto, es muy importante reconocer los signos radiológicos característicos de cada una en las diversas modalidades imagenológicas para poder sospechar y orientar el diagnóstico.

Abstract Non-atheromatous arterial pathology constitutes a group of infrequent pathologies but of great importance due to their associated morbidity and mortality. In general, the clinical presentation of these pathologies is quite nonspecific, so the clinician decides to perform imaging studies thinking of more common entities. It is for this reason that it is very important to recognize characteristic radiological signs in the various imaging modalities to be able to suspect and orient a pathology of this kind.

PET-CT FDG en el diagnóstico y seguimiento de vasculitis de grandes vasos / Positron emission tomography-CT scan (PET-CT) in the diagnosis of large vessel vasculitis

Vasculitides are a broad group of diseases that can involve any kind of vessel in any organ. These can be classified according to the size of the affected vessels. The most used classification categorizes them in small, medium, and large vessel vasculitis. Large vessel vasculitis can be further divided in Takayasu arteritis and giant cell arteritis which can sometimes be indistinguishable, even with biopsy. Radiology plays an important role identifying distribution patterns and disease extension18. Fluorine-Fluorodeoxyglucose (FDG) PET-CT shows increased vessel wall FDG uptake in patients with active large vessel vasculitis. Multiple studies show that FDG PET-CT helps to identify the anatomic structures with the disease, as well as evaluate its progression with a high sensibility and specificity in non-treated patients with large vessel vasculitis.

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

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

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

Vasculitis del tronco celíaco como presentación de arteritis de Takayasu / Vasculitis of the celiac artery as a presentation of Takayasu's arteritis

La arteritis de Takayasu es una vasculitis idiopática y granulomatosa crónica que se manifiesta en forma de panaortitis, de etiología desconocida, aunque se postula un origen autoinmune. Es progresiva y genera, tanto en adultos como en niños, estenosis segmentaria, oclusión, dilatación y/o aneurismas. La vasculitis aislada gastrointestinal sin afectación sistémica es rara. Este caso lleva a tener en cuenta la manifestación abdominal atípica de la arteritis de Takayasu en el diagnóstico diferencial de un síntoma frecuente, como la epigastralgia, y a destacar el rol que ocupan en la actualidad los métodos de imágenes no invasivos para su diagnóstico.

Takayasu arteritis is an idiopathic and chronic granulomatous vasculitis manifested in the form of panaortitis, of unknown etiology, even though an autoimmune origin is postulated. It is progressive and generates, in adults and children, segmental stenosis, occlusion, dilation and / or aneurysms. Isolated gastrointestinal vasculitis without systemic involvement is rare. This case leads us to take into account the atypical abdominal manifestation of Takayasu arteritis in the differential diagnosis of a frequent symptom, such as epigastralgia, and to highlight the role currently played by non-invasive imaging methods for its diagnosis.

Taquicardia persistente en mujer joven deportista / A young woman with persistent tachycardia

Resumen: Una mujer joven, deportista, evoluciona con compromiso del estado general y síntomas constitucionales, asociado a taquicardia persistente y baja de peso de casi un año de evolución, con laboratorio, electrocardiograma y ecocardiograma inicialmente anodinos.

Abstracts: An athletic young woman presented with almost one year of general symptoms, weight loss, and persistent tachycardia. The initial laboratory, electrocardiogram and echocardiogram findings were unremarkable.

Arteritis de Takayasu con pulmonares estenosis severa de ramas principales / Takayasu's arteritis with severe stenosis of main pulmonary artery branches

A 40-year-old woman consulted for progressive dyspnea. Physical examination revealed systolic murmurs in the upper thoracic area and asymmetric pulses in the upper extremities. Echocardiography revealed a 117-mmHg systolic gradient across the tricuspid valve and CT angiography revealed severe stenosis of pulmonary artery branches. A discussion of the disease is included.

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

ABSTRACT Objective: To evaluate 18F-fluorodeoxyglucose (18F-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 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 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 18F-FDG uptake in arterial wall are associated with active TA.

RESUMO Objetivo: Avaliar a captação de 18F-fluordesoxiglicose (FDG) na tomografia por emissão de pósitrons – tomografia computadorizada (PET-CT) – e os níveis séricos de diferentes citocinas e da metaloproteinases da matriz (MMP) em pacientes com arterite de Takayasu (AT) e associações com a atividade da doença. Métodos: Foram mensurados os níveis séricos do fator de necrose tumoral-α (TNF-α), interleucina (IL)-2, IL-6, IL-8, IL-12, IL-18, MMP-3 e MMP-9 em 36 pacientes com AT e 36 controles. O valor padronizado de captação máximo (SUVmax) de 18F-FDG nas paredes arteriais foi determinado por exames de PET-CT. Os pacientes com AT foram classificados como doença ativa, doença inativa e possível doença ativa. Resultados: Os níveis séricos de IL-6 e MMP-3 foram mais altos em pacientes com AT do que nos controles (p < 0,001). Os níveis séricos de IL-6 foram mais elevados em pacientes com doença ativa e em pacientes com possível doença ativa do que naqueles com doença inativa (p < 0,0001). Os pacientes com doença ativa apresentaram níveis séricos mais elevados de TNF-α do que os pacientes com doença inativa (p = 0,049), enquanto os indivíduos com possível doença ativa apresentaram maiores níveis séricos de IL-18 do que os pacientes com doença inativa (p = 0,046). Aqueles com doença ativa apresentaram maiores valores de SUVmax do que aqueles com doença inativa (p = 0,042). De acordo com a curva ROC, o SUVmax foi capaz de predizer a doença ativa na AT e valores ≥ 1,3 estavam associados à atividade da doença (p = 0,039). Os níveis séricos de TNF-α foram maiores em pacientes com SUVmax ≥ 1,3 do que naqueles com valor < 1,3 (p = 0,045) e controles (p = 0,012). Os níveis séricos de IL-6 foram mais elevados em pacientes com SUVmax ≥ 1,3 do que nos controles (p < 0,001). Não foram encontradas diferenças em relação a outros biomarcadores entre pacientes com AT e controles. Conclusões: Níveis séricos elevados de IL-6 e TNF-α, bem como uma maior captação arterial de 18F-FDG, estão associados à AT ativa.

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.

Posterior reversible encephalopathy syndrome revealing Takayasu's arteritis.

Posterior reversible encephalopathy syndrome is a rare neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging predominantly affecting the posterior regions of the brain. We report a 10-years-8-months-old girl who presented with headache, multiple seizures, and altered sensorium. Her blood pressure was 130/100 mmHg and left brachial pulse was not palpable. CT scan brain showed typical non-enhancing hypodensities in bilateral parieto-occiptal lobes. Prompt treatment of the hypertension led to rapid reversal of neurological symptoms. CT aortogram revealed aortoarteritis with bilateral renal artery stenosis.

Takayasu's Arteritis Treated by Percutaneous Transluminal Angioplasty with Stenting in the Descending Aorta

A 17-yr-old young woman was referred to our hospital with a 2-yr history of claudication of the lower extremities and severe arterial hypertension. Physical examination revealed significantly different blood pressures between both arms (160/92 and 180/95 mmHg) and legs (92/61 and 82/57 mmHg). The hematological and biochemical values were within their normal ranges, except for the increased erythrocyte sedimentation rate (83 mm/hr) and C-reactive protein (6.19 mg/L). On 3- dimensional computed tomographic angiography, the ascending aorta, the aortic arch and its branches, and the thoracic and, descending aorta, but not the renal artery, were shown to be stenotic. The diagnosis of type IIb Takayasu's arteritis was made according to the new angiographic classification of Takayasu's arteritis, Takyasu conference 1994. Percutaneous transluminal angioplasty with stenting was performed on the thoracic and abdominal aorta. After the interventional procedures, the upper extremity blood pressure improved from 162/101 mmHg to 132/85 mmHg, respectively. She has been free of claudication and there have been no cardiac events during 2-yr of clinical follow-up.

Hemorrhagic Colitis due to Escherichia coli O157 Infection in a Patient with Takayasu's Arteritis / 대한소화기학회지

Escherichia coli (E coli) O157 may cause abdominal pain and diarrhea followed by hematochezia. Most of cases resolve spontaneously after several days. Takayasu's arteritis affects medium- and large-sized arteries, aortic arch and its branch, and rarely affects inferior mesenteric artery. In case of Takayasu's arteritis with hematochezia, we must distinguish among ulcerative colitis, ischemic colitis, and infectious colitis with Takayasu's arteritis. We report a case of 17-year-old woman who suffered from hemorrhagic colitis by E. coli O157, and combined with leg claudication and abdominal pain by Takayasu's arteritis that affected abdominal aorta and inferior mesenteric artery. Sigmoidoscopy showed edematous, hyperemic mucosa and superficial ulcerations in the sigmoid colon. Abdominal CT scan showed diffuse submucosal edema, narrowing of distal abdominal aorta and inferior mesenteric artery. Hematochezia disappeared after the conservative treatment and leg claudication and abdominal pain disappeared after the aortic angioplasty.

Follow-up CT Evaluation of the Mural Changes in Active Takayasu Arteritis

OBJECTIVE: We wanted to evaluate the mural changes by CT on the follow-up examination of patients with active Takayasu arteritis. MATERIALS AND METHODS: The study included 18 patients, (4 males and 14 females), with active Takayasu arteritis. A total of 44 CT examinations were done during the follow-up period (mean: 55.6 months). At the time of the last follow-up CT, the disease, on the basis of the erythrocyte sedimentation rate (ESR), was found to be inactive in five patients and the disease was active and persistent in 13 patients. The thickness and CT attenuation of the aortic wall on the precontrast, arterial and venous phases were measured on the initial and the follow-up CT examinations. The ratio of the mural attenuation over that of the back muscle on the initial CT was compared with the ratio found on the follow-up CT. RESULTS: The initial CT findings included high density and calcifications of the aortic wall in the precontrast images and a thickened wall with enhancements in the arterial and the venous phases. A low-attenuation ring was demonstrated in the venous phase in 15 patients (83%). On the follow-up evaluation, the mean mural thickness decreased significantly from 4.1 mm to 2.4 mm. The mean mural attenuation ratio in the venous phase decreased significantly from 1.9 to 1.3 (p = 0.001). The low attenuation ring was identified in seven patients (39%) who had only with active, persistent Takayasu arteritis. CONCLUSION: The mural changes demonstrated by the follow-up CT evaluations for the patients with active Takayasu arteritis included a decrease of the mural thickness and enhancement, disappearance of the low-attenuation ring on the venous phase, and an increase of the mural attenuation and calcification on the precontrast phase.

Surgical Management of Takayasu's Arteritis

The results of surgical bypass and endarterectomy in Takayasu's arteritis (TA) were reported to be poor compared to usual atherosclerosis patients. However, if ischemic symptoms due to occlusive disease were severe, surgical procedures were inevitable. We report surgical experience of 5 patients with TA. Five women (ranged from 26 to 58 yr) were operated between June 1998 and May 2004. Three patients showed occlusion of main branches of aortic arch and had symptoms of cerebral ischemia. One patient showed near total occlusion in the midabdominal aorta and had symptoms of orthopnea and uncontrolled hypertension. One patient showed total occlusion of abdominal aorta at the level of aortic bifurcation and had a symptom of severe claudication on both legs. Bypasses from the ascending aorta to the carotid artery were performed in 3 cases. Bypass from the thoracic aorta to the left common iliac artery was performed in one case and endarterectomy of abdominal aorta in one case. The ischemic symptoms related with arterial occlusion were resolved after surgery. And the symptoms of cardiac failure disappeared. The symptomatic TA frequently required arterial reconstruction. The symptomatic improvement and excellent mid-term patency could be expected after arterial reconstruction and endarterectomy.

Recent advances in the management of non-specific aorto-arteritis.

Non-Specific Aorto-arteritis or Takayasu's arteritis (TA) is a chronic inflammatory disease of the aorta and its major branches which either presents with constitutional symptoms in its active phase or with steno-occlusive symptoms in the healing phase. In children, it is an aggressive disease and often lethal. Congestive heart failure is more common in children than adults. TA often goes undiagnosed till it is too late and death rate of 35% or more is observed in 5 years. Therapeutic Interventional Cardiology offers new hope and saves the children with TA from certain death. Today, percutaneous transluminar balloon angioplasty (PTBA) has emerged as a non-surgical, safe, effective alternative therapeutic option and endovascular stents have revolutionised the management. The disease must be suspected and diagnosed precisely with echocardiography and angiography before it is too late to manage.