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.

Arterite de Takayasu: diagnóstico, evolução e tratamento de paciente com ulceração em couro cabeludo. Relato de caso / Takayasu's arteritis: diagnosis, evolution and treatment of patient with scalp ulceration. Case report

A arterite de Takayasu é uma vasculite autoimune rara, que afeta predominantemente a aorta e seus ramos principais, produzindo variados sinais e sintomas, como febre, mialgia, hipertensão, úlceras e isquemia. É mais comum na Ásia e menos frequente nos países ocidentais, acometendo mulheres em idade reprodutiva. As manifestações cutâneas podem estar presentes. Os critérios diagnósticos do American College of Rheumatology incluem idade <40 anos, diminuição dos pulsos braquiais, claudicação de extremidades, diferença de 10mmHg na pressão arterial sistólica de membros superiores, sopros em suclávia e aorta, e alterações angiográficas de aorta e seus arcos principais. Os melhores resultados terapêuticos baseiam-se na corticoterapia associada à imunossupressores como metotrexato. O uso de imunobiológicos é incipiente e carece de estudos para comprovar sua eficácia. A paciente em questão apresentava-se com quadro atípico, manifestando ulceração em couro cabeludo, decorrente de estenose em ramos superficiais da carótida externa. Evoluiu com trombose venosa cerebral e osteomielite, sendo submetida à oxigenoterapia hiperbárica e imunossupressão com 160mg de prednisona.Velocidade de hemossedimentação e proteína C-reativa estavam elevados, mas seu valor isolado carece de especificidade, embora sejam critérios de atividade da doença. A terapêutica com prednisona, metotrexato e anticoagulação com varfarina foi eficiente, pois reduziu a área da lesão em couro cabeludo e minimizou o quadro sistêmico, propiciando melhor qualidade de vida para a paciente.

Takayasu's arteritis is a rare autoimmune vasculitis, which primarily affects the aorta and its main branches, producing varied symptoms and signs, such as fever, myalgia, hypertension, ulcers, and ischemia. It is more common in Asia and less frequente in western countries, affecting women of childbearing age.Cutaneous manifestations may be present. The diagnostic criteria of the American College of Rheumatology include age <40 years,reduction of brachial pulses, extremity claudication, difference of 10 mmHg in systolic blood pressure of upper limbs, murmurs in subclavian artery and aorta, and angiographic changes of the aorta and its main arches. The best therapeutic results are based on corticosteroid therapy associated with immunosuppressants such as methotrexate. The use of immunobiologicals is incipiente and requires studies to prove its effectiveness. The patient presented with an atypical clinical picture, showing ulceration on the scalp as a result of stenosis in superficial branches of the external carotid. She progressed with cerebral venous thrombosis and osteomyelitis, undergoing hyperbaric oxygen therapy and immunosuppression with 160mg of prednisone. Erythrocyte sedimentationrate and C-Reactive Protein were high, but their individual value lacks specificity, although they are criteria for disease activity. Therapy with prednisone, methotrexate, and anticoagulation with warfarin were efficient, because they reduced the area of the lesion on the scalp, and minimized the systemic picture, providing better quality of life for the patient.

Hipertensión arterial como presentación de arteritis de Takayasu / Hypertension as presentation of Takayasu arteritis

Se presentó un paciente de 19 años con hipertensión arterial, de 3 años de evolución, en tratamiento, y aparición reciente de sensación de fatiga muscular en miembros inferiores a la marcha. En la exploración era significativa la existencia un soplo holosistólico III/VI del borde esternal izquierdo, más evidente hacia la base aunque perceptible en todo el área cardíaca y la gran asimetría de pulsos y tensión arterial entre miembros superiores e inferiores. Se halló que cumplía los criterios diagnósticos para arteritis de Takayasu del Colegio Americano de Reumatología incluido el angiográfico. Se comprobó la existencia de estrechamiento concéntrico de la aorta torácica, el cual se origina casi inmediatamente distal al origen de la arteria subclavia izquierda y se extendía al segmento abdominal hasta la altura de la emergencia de ambas arterias renales, correspondiente al tipo III de la clasificación de Hata. El paciente se egresó en tratamiento con 60 mg de prednisona por día, terapia antihipertensiva y evaluación periódica ambulatoria. Aunque existen reportes previos, es infrecuente en la práctica clínica esta forma de presentación de arteritis de Takayasu como pseudocoartación que produce hipertensión arterial por lo que consideramos de utilidad su notificación.

A 19 year-old patient with hypertension is presented with 3 years of evolution, treatment, and recent onset of muscle fatigue sensation in his lower limbs. On examination it was significant the presence of a holosystolic murmur III/VI at the left sternal border, although more evident towards perceptible basis throughout the cardiac area and the large asymmetry in pulse and blood pressure between upper and lower limbs. It was found this patiente met the diagnostic criteria for Takayasu arteritis from the American College of Rheumatology including angiography. It was found the existence of concentric narrowing of the thoracic aorta, which originates almost immediately distal to the origin of the left subclavian artery and the abdominal segment extended to the height of the emergence of both renal arteries, corresponding to Hata classification type III. This patient was discharged on treatment with 60 mg of prednisone per day, antihypertensive therapy and outpatient periodic evaluation. Although there are previous reports, this form of presentation of Takayasu arteritis it is not uncommon in clinical practice, as pseudocoarctation producing hypertension, so this report is considered useful.

Arterite de Takayasu / Takayasu's arteritis

O presente caso refere-se a uma menina de 15 anos de idade que apresentava febre e anemia durante seis meses, acompanhadas de claudicação do membro superior direito e sopro abdominal em região do epigástrio.

The present case refers to a 15 years-old girl that showed symptoms of fever and anemia during six months, followe of lameness of the right shoulder and abdominal blow in region of epigastrium.

Arteritis de Takayasu y embarazo / Takayasu arteritis and pregnancy

La arteritis de Takayasu es una enfermedad vascular inflamatoria crónica e inespecífica, de etiología desconocida, que usualmente afecta las ramas del arco aórtico y se presenta con una alta incidencia durante la edad reproductiva. El estado de la enfermedad al comienzo del embarazo es un factor decisivo para establecer el manejo ulterior. De la vigilancia multidisciplinaria dependerán en gran medida los resultados materno-fetales favorables. Presentamos el caso de una gestante de 20 años con diagnóstico de arteritis de Takayasu realizado un año previo a la concepción y que cursó un embarazo y parto sin complicaciones bajo seguimiento, en el servicio de obstetricia del Hospital general docente "Enrique Cabrera". Se revisó la literatura sobre el tema y su relación con el embarazo realizando una breve exposición de las características clínicas, diagnóstico, manejo y pronóstico de la enfermedad

Takayasu's arteritis is a nonspecific, chronic, inflammatory and vascular disease, its etiology is unknown usually occluding one or more of the large branches of the aortic arch, with a high incidence during reproductive age. Disease condition at pregnancy onset is a decisive factor for its further management. From multidisciplinary surveillance will depend in large extent the favorable maternal-fetal result. This is the case of a pregnant aged 20 diagnosed with Takayasu's arteritis during one year prior to conception and with a complications-free pregnancy and labor under follow-up in Obstetrics Service of "Enrique Cabrera" Hospital. Literature on this matter was reviewed and its relation to pregnancy, with a brief exposition of clinical features, diagnosis, management and disease prognosis

Takayasu's arteritis and Crohn's disease: an unusual association

A arterite de Takayasu e a doença de Crohn são doenças inflamatórias com etiologia desconhecida. Raramente ocorrem de modo concomitante em um mesmo indivíduo, havendo menos de 30 casos relatados na literatura. Este trabalho descreve essa associação em uma paciente de 36 anos de idade portadora de doença de Crohn, que apresentou redução dos pulsos no membro superior esquerdo e pressão arterial de 60/40 mmHg. A angiotomografia evidenciou estenose segmentar de artéria subclávia esquerda e espessamento parietal circunferencial da aorta na transição toracoabdominal entre T10 e L1, estabelecendo o diagnóstico de arterite de Takayasu. Ambas as patologias são mediadas imunologicamente e apresentam granulomas e vasculite granulomatosa, o que contribui para reforçar a hipótese de uma origem imunológica comum no seu desenvolvimento. Acreditamos que este seja o primeiro caso relatado na literatura brasileira da presença concomitante destas duas enfermidades.

Takayasu's arteritis and Crohn's disease are chronic inflammatory diseases with unknown etiology. They rarely occur together in the same individual, with less than 30 cases reported in the literature. This case report describes this association in a 36-year-old woman with Crohn's disease and weak pulses in her left arm with blood pressure of 60/40 mmHg. Angiotomography showed segmental stenosis in the left subclavian artery and circumferential thickening of the aortic wall between T10 and L1, establishing the diagnosis of Takayasu's arteritis. Both are organ-specific and immune-mediated diseases and exhibit granulomas and granulomatous vasculitis, which contribute to reinforce the hypothesis of a common immunologic origin. We believe that this is the first case of concomitant presence of these two diseases reported in the Brazilian literature.

An experimental design for induction of non-specific aortoarteritis.

BACKGROUND: An attempt was made to induce aortoarteritis in mice by using various antigens. METHODS AND RESULTS: The Swiss mice were immunized with eight different antigens and were grouped A to G. Group H served as control. The mice were then bled at 1st, 2nd, 4th, 6th and 8th month interval post-immunization for estimating antibody titer. Then the mice were sacrificed and the heart, aorta and kidney were taken out and processed for hematoxylin-eosin staining. There was gradual increase in the antibody titer from 1st month till 4th month within all the experimental groups (A-G), when compared with control group H. The titer started falling sharply from 6th month post-immunization. However, the control group H did not show much variation. When each individual group was compared separately with control group H, the significant statistical value was obtained. Histopathological examination revealed mild inflammation (+) in kidney by 2nd month, moderate inflammation (++) by 6th month, extensive inflammation (+++) by 8th month and alteration in the normal parenchyma of kidney by 8th month. CONCLUSIONS: The histopathological changes brought out through antigens were more pronounced by 8th month following injection of tunica media, tunica adventitia, tunica intima and aorta collagen as compared to that of standard collagen and mouse aorta injections.

Artritis de Takayasu y embarazo / Takayasu's arteritis and pregnancy

La enfermedad de Takayasu es una enfermedad granulomatosa crónica de etiología desconocida que se caracteriza por arteritis y obstrucción secundaria de la aorta y sus grandes ramas. Se presenta predominantemente en mujeres en edad fértil por lo que su asociación con embarazo se ve ocasionalmente. Durante su fase aguda constituye un difícil diagnóstico diferencial de todo cuadro febril prolongado con compromiso del estado general, VHS elevada e hipergamagiobulinemia, destacando en su fase tardía los síntomas y signos derivados de insuficiencia vascular. Su asociación con el embarazo es de gran relevancia por cuanto se ha asociado a hipertensión arterial, insuficiencia cardíaca, retardo de­ crecimiento intrauterino y parto prematuro. Se presenta un estudio retrospectivo de 7 embarazos manejados en la unidad de medicina perinatal de la Universidad Católica de Chile entre los años 1985-1995. Se analiza la morbilidad materna y el resultado perinatal