Enfermedad relacionada con IgG4, enfermedad de Erdheim Chester y compromiso aórtico y/o sus ramas. Diagnóstico diferencial con otras vasculitis de grandes vasos / IgG4 related disease, Erdheim Chester disease and aortic involvement and/or its branches. Differential diagnosis with other large vessel vasculitis

La afección cardiovascular en entidades como Erdheim-Chester (EEC), una rara histiocitosis de células no Langerhans, y la enfermedad relacionada con IgG4 (ER-IgG4), una afección fibrinoinflamatoria inmunomediada, es muy variada y habitualmente asintomática hasta su progresión a daños irreversibles cuando no es sospechada. Ante la dificultad de realizar biopsias por el sitio anatómico, es fundamental valerse de características clínicas, demográficas o imagenológicas que puedan diferenciarlas de otras entidades, como las arteritis de grandes vasos. La población masculina, los mayores de 60 años con compromiso a nivel de la aorta abdominal infrarrenal o aneurismas a nivel de la aorta ascendente con o sin compromiso de otros órganos, son orientativos de ER-IgG4. En la EEC es característico el tejido blando concéntrico que recubre la aorta (aorta recubierta) y sobre todo ante la presencia de fibrosis retroperitoneal, compromiso de huesos largos, hidronefrosis, lesión renal aguda posrrenal e hipertensión arterial, existencia de tejido fibrótico perirrenal, engrosamiento de la fascia renal y tejido adiposo perirrenal (signo del riñón peludo).

Cardiovascular disease in entities such as Erdheim-Chester (ECD), a rare non-Langerhans cell histiocytosis, and IgG4-related disease (ER-IgG4), an immunemediated fibrinoinflammatory disease, is highly varied and usually asymptomatic until it progresses to irreversible damage if they are not taken into account. Given the difficulty of performing biopsies by the anatomical site, it is essential to use clinical, demographic or imaging characteristics that can differentiate them from other entities such as large vessel arteritis. Male population, over 60 years of age with involvement of the infrarenal abdominal aorta or aneurysms at the level of the ascending aorta with or without involvement of other organs, are indicative of ER-IgG4. In ECD, the concentric soft tissue covering the aorta (covered aorta) is characteristic, and especially in the presence of retroperitoneal fibrosis, involvement of long bones, hydronephrosis, post-renal acute kidney injury and arterial hypertension, the presence of perirenal fibrotic tissue, thickening of the renal fascia, perirenal adipose tissue (hairy kidney sign).

Aneurisma aórtico e insuficiencia valvular severa por sífilis terciaria en paciente joven, una enfermedad desatendida / Aortic aneurysm and severe valvular insufficiency due to tertiary syphilis in a young patient, a neglected disease / Aneurisma da aorta e insuficiência valvular grave devido à sífilis terciária em um paciente jovem, uma doença negligenciada

La sífilis es causada por Treponema pallidum, es reconocida por la variedad de sus manifestaciones clínicas; compromete el sistema nervioso, tegumentario y cardiovascular. Las alteraciones cardiovasculares ocurren en 70% de los casos con sífilis no tratada, el 71% de las aortitis sifilíticas desarrollan aneurismas aórticos y la complicación más frecuente es la insuficiencia aórtica. El tratamiento va dirigido a la enfermedad subyacente, con manejo antibiótico para la sífilis terciaria y las complicaciones tromboembólicas y si requiere reparación quirúrgica. Presentamos un caso poco frecuente de un paciente diagnosticado de infección por el virus de la inmunodeficiencia humana y complicaciones cardiovasculares típicas de la sífilis terciaria, que precisó tratamiento médico y quirúrgico, que fue exitoso. Destacamos la pericia clínica de los profesionales de nuestra institución para diagnosticar e identificar las complicaciones cardiovasculares de la sífilis terciaria.

Syphilis is caused by Treponema pallidum and is recognized by the variety of its clinical manifestations; it involves the nervous, integumentary and cardiovascular systems. Cardiovascular alterations occur in 70% of cases with untreated syphilis, 71% of syphilitic aortitis develop aortic aneurysms and the most frequent complication is aortic insufficiency. Treatment is directed at the underlying disease, with antibiotic treatment for tertiary syphilis and thromboembolic complications and if necessary surgical repair. We present a rare case of a patient diagnosed with human immunodeficiency virus infection and cardiovascular complications typical of tertiary syphilis, who required medical and surgical treatment, which was successful. We highlight the clinical experience of our institution's professionals in the diagnosis and identification of cardiovascular complications of tertiary syphilis.

A sífilis é causada pelo Treponema pallidum e é reconhecida pela variedade de suas manifestações clínicas; ela afeta os sistemas nervoso, tegumentar e cardiovascular. As alterações cardiovasculares ocorrem em 70% dos casos de sífilis não tratada, 71% dos casos de aortite sifilítica desenvolvem aneurismas aórticos e a complicação mais frequente é a insuficiência aórtica. O tratamento é direcionado para a doença subjacente, com tratamento antibiótico para sífilis terciária e complicações tromboembólicas e, se necessário, reparo cirúrgico. Apresentamos um caso raro de um paciente diagnosticado com infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que precisou de tratamento médico e cirúrgico, que foi bem-sucedido. Apresentamos um caso raro de um paciente com diagnóstico de infecção pelo vírus da imunodeficiência humana e complicações cardiovasculares típicas da sífilis terciária, que necessitou de tratamento médico e cirúrgico, o qual foi bem-sucedido. Destacamos a experiência clínica dos profissionais de nossa instituição no diagnóstico e na identificação das complicações cardiovasculares da sífilis terciária.

Clinicopathological features of twenty four active aortitis cases / 中华风湿病学杂志

Objective:To explore the clinicaland pathological characteristics of aortitisin order to improve the understanding of this rare conditionand improve correct diagnostic rate.Methods:Twenty-four cases of active aortitis were identified from a total of 1 838 cases of ascending aorta specimens in the last 6 years at Wuhan Asia General Hospital. Clinical data including medical history, laboratory and imaging data were collected and the treatment with immunosuppressive and hormonal treatment as well as follow-up data were analyzed. Pathological data including gross findings, microscopic features of the aortic valve and aorta were analyzed. Continuous variables were expressed as mean±standard deviation. Frequencies were described as percentages. Results:Among the 24 cases of active aortitis, 1 case was clinically diagnosed as aortitis before operation, 7 cases were suspected aortitis before operation, and the other 16 cases were diagnosed as aortitis after pathological examination. Among those 16 cases, one case was Behcet′s syndrome, 2 cases were infectious aortitis, 3 cases were Takayasu aortitis, and 10 cases were clinically isolated aortitis. None case had aortic stenosis, while 21 cases had aortic valve insufficiency. Eleven cases of aortitis showed coagulation necrosis. In the 5 cases of Behcet′s syndrome, 3 had acute noninfectious endocarditis of aortic valve.Conclusions:Most of the aortitis in this study was found accidentally in pathological examination. All of the clinically isolated aortitiswere misdiagnosed before pathological examination. Most of the patients with aortitis had simple aortic valve insufficiency. Coagulation necrosis is an important clue for the diagnosis of aortitis. Acute noninfectious endocarditis is an important clue for the diagnosis of Behcet′s syndrome.

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

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

Aneurisma aórtico abdominal infeccioso por Salmonella en un paciente con diabetes mellitus / Infected abdominal aortic aneurysm due to Salmonella in a patient with diabetes mellitus

Resumen Presentamos el caso de un paciente de edad avanzada, con diabetes mellitus descompensada, quien presentó un cuadro clínico de fiebre y dolor abdominal recurrente, tras lo cual fue diagnosticado con un aneurisma infeccioso de la aorta abdominal, los cuales representan solamente un 1% de todos los aneurismas. El paciente fue sometido a resección quirúrgica del aneurisma, injerto con dacrón impregnando con rifampicina y tratamiento antibiótico intravenoso. La microbiología del aneurisma confirmó infección por Salmonella. Actualmente, el paciente se encuentra asintomático y sin evidencia laboratorial de proceso inflamatorio.

Abstract We present a case of an elderly patient with uncontrolled diabetes mellitus, who presented with recurrent fever and abdominal pain, after which he was diagnosed with an infected abdominal aortic aneurysm, which represents only 1% of all aneurysms. The patient underwent surgical resection of the aneurysm, rifampicine-impregnated Dacron graft placement and intravenous antibiotic treatment. Microbiology reported Salmonella infection in the aneurysm. Currently, the patient is asymptomatic and without laboratory evidence of inflammatory process.

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.

Aneurisma de la aorta abdominal infectado por Campylobacter fetus: comunicación de un caso clínico y revisión de la literatura médica / Abdominal aortic aneurysm infected with Campylobacter fetus: case report and medical literature review

Resumen La infección es una complicación infrecuente de los aneurismas de la aorta abdominal. Campylobacter fetus tiene un especial tropismo por el endotelio vascular y ha sido reportado como causa de infección de aneurismas aórticos. Este tipo de infección es de alta mortalidad por lo que el reconocimiento temprano con el inicio precoz de terapia antibacteriana efectiva es clave. Además del tratamiento médico, puede requerirse cirugía, la que tiene una alta letalidad en pacientes inestables y con comorbilidades. Comunicamos el caso clínico de un adulto mayor con un aneurisma de aorta abdominal infectado por C. fetus. Dado su compromiso del estado general y antecedentes cardiovasculares se decidió tratamiento médico con imipenem, con una buena respuesta clínica y microbiológica, sin recurrencia de los síntomas. También se presenta una revisión de los casos publicados.

Abstract Infection is a rare complication of abdominal aortic aneurysms. Campylobacter fetus has special tropism for vascular endothelium. It has been reported as a cause of infected abdominal aortic aneurysms. The mortality of these patients is high, so an early recognition with a start of antibiotic therapy is crucial. In addition to medical treatment surgery may be required, which has high mortality in patients with many diseases and unstable. We report the case of an old man with infected abdominal aortic aneurysm with C. fetus, whom was decided to be treated with imipenem, due to his cardiovascular history and his general condition at admission. The patient showed a good clinical response without recurrence of symptoms. We also carry out a review of the reported cases.

Reparación de aneurisma de aorta torácica con compromiso de vasos del cuello en paciente con sífilis terciaria. Reporte de caso / Thoracic aortic aneurysm repair involving neck vessels in a patient with tertiary syphilis. Case report

RESUMEN La dilación aneurismática de la aorta es una patología con una prevalencia importante entre las afecciones que aquejan a la misma (dependiendo de la zona que compromete) sea aorta torácica o abdominal presenta una mortalidad variable que exige distintas y específicas formas de reparación acorde a la factibilidad técnica y la fragilidad del paciente. La angiografía por tomografía computada o por resonancia magnética son los exámenes de elección para el diagnóstico diferencial de las distintas patologías de la aorta, aportando detalles estructurales para trazar estrategias en relación a técnicas a emplear para su abordaje o reparación. Entre las múltiples opciones terapéuticas para las dilataciones aneurismáticas que comprometen a la aorta torácica, la reparación endovascular de los aneurismas se han convertido en una opción terapéutica muy viable para proporcionar una menor comorbilidad frente a la reparación quirúrgica a cielo abierto, aun en situaciones muy complejas en donde pudieran coexistir un compromiso de ramas aórticas importantes, en estos casos la reparación endovascular de los aneurismas continua siendo de elección al poder ser complementada con una revascularización quirúrgica mínima en forma previa o concomitante, constituyéndose así en una técnica híbrida eficaz y eficiente. El actual trabajo presenta a un paciente con el diagnóstico de sífilis terciaria en quien se evidencia un compromiso importante de la aorta torácica, consistente en una dilatación aneurismática en su porción descendente que comprime extrínsecamente el nacimiento de las arterias: carótida primitiva y subclavia izquierda. Se realizó en un primer tiempo un puente carótido-carotídeo y en un segundo tiempo el tratamiento endovascular consistente en el implante de una endoprótesis por vía femoral hasta la lesión en la aorta torácica, los mismos sin compromiso de la circulación de los vasos del cuello preservando la indemnidad de la circulación cerebral y lográndose una exclusión exitosa del aneurisma torácico con la endoprótesis.

ABSTRACT The aneurysmal dilation of the aorta is a pathology with a significant prevalence among the conditions that afflict it, depending on the area involved, whether thoracic or abdominal aorta has a variable mortality that requires different and specific forms of repair according to the technical feasibility and fragility of the patient. Computed tomography angiography or magnetic resonance angiography are the tests of choice for the differential diagnosis of the different pathologies of the aorta, providing structural details to design strategies in relation to techniques to be used for its approach or repair. Among the multiple therapeutic options for aneurysmal dilatations involving the thoracic aorta, endovascular repair of aneurysms have become a very viable therapeutic option by providing less comorbidity compared to open surgical repair, even in very complex situations where important aortic branches may coexist, in these cases endovascular repair of aneurysms continues to be of choice as it can be complemented with minimal surgical revascularization either previously or concomitantly, thus constituting an effective and efficient hybrid technique. This work presents a patient with the diagnosis of tertiary syphilis in whom a significant compromise of the thoracic aorta is evidenced, consisting of an aneurysmal dilatation in its descending portion that extrinsically compresses the origin of the arteries: primitive carotid and left subclavian. A carotid:carotid bridge was performed firstly and in the femoral route until the lesion in the thoracic aorta, the same without compromising the circulation of the vessels of the neck preserving the indemnity of the cerebral circulation and achieving a successful exclusion of the thoracic aneurysm with the endoprosthesis.

Aortitis sifilítica: a propósito de un caso del sur colombiano / Syphilitic aortitis: about a case in South Colombia

Resumen Se expone el caso de una paciente de 73 años procedente y residente de Florencia, Caquetá (Colombia), con antecedentes de hipertensión arterial, que ingresa a un hospital de alta complejidad por un cuadro clínico de 3 meses de evolución consistente en dolor torácico tipo opresivo en la región anterior izquierda del tórax, no irradiado, de intensidad 8/10. En el ecocardiograma se evidencia enfermedad calcificada de la válvula aórtica con estenosis aortica grave, insuficiencia aórtica, hipertrofia del ventrículo izquierdo e insuficiencia mitral leve. Dentro de los estudios prequirúrgicos presentó VDRL con 1:4 diluciones, se confirmó el diagnóstico con el resultado de la prueba treponémica y se descartaron otras enfermedades infecciosas. Se realizó remplazo valvular con bioprótesis y tubo valvular supracoronario. En la biopsia se confirmó la valvulitis crónica cicatricial con extensas calcificaciones. Posterior a la intervención quirúrgica se dio egreso por adecuada evolución clínica.

Abstract The case of a 73-year-old female patient obtained and resident of Florencia, Caquetá (Colombia), with a history of hypertension who is admitted to a highly complex hospital due to a 3-month clinical picture of constant evolution in pain is presented. Thoracic oppressive type in the anterior region of the left thorax, non-irradiated, of intensity 8/10. The echocardiogram shows calcified aortic valve disease with severe aortic stenosis, aortic regurgitation, left ventricular hypertrophy, and mild grade mitral regurgitation. Within the pre-surgical studies, she presented VDRL with 1:4 dilutions and the diagnosis was confirmed with the result of the treponemal test and other infectious diseases were ruled out. Valvular replacement was performed with a bioprosthesis plus a supra-coronary valvular tube. In the biopsy, chronic scar valvulitis with extensive calcifications was confirmed. After the surgical intervention, discharge was performed due to adequate clinical evolution.

Aortitis sifilítica: manifestación olvidada de la sífilis / Syphilitic aortitis: forgotten manifestation of syphilis

Resumen La sífilis es una infección bacteriana producida por el Treponema pallidum (espiroqueta). Consta de varias etapas: primaria, secundaria y terciaria, según el tiempo de evolución desde el momento en que se adquiere la infección. Cada etapa comprende diferentes manifestaciones clínicas; los síntomas cardiovasculares forman parte de la sífilis terciaria, en cuyo caso la aortitis sifilítica es la principal forma de presentación. Se expone el caso de un paciente que consultó por déficit neurológico focal, en quien por medio de estudios de extensión se documentó neurosífilis e insuficiencia valvular aórtica severa secundaria a perforación de la válvula coronaria derecha, que requirió recambio valvular aórtico por bioprótesis. Adicionalmente, se hace una revisión de las principales manifestaciones cardiovasculares de esta enfermedad. Aunque en la era postantibiótica este tipo de manifestaciones tardías son cada vez menos frecuentes, es imperativo conocerlas.

Abstract Syphilis is a bacterial infection caused by Treponema pallidum (spirochete). It has various stages: primary, secondary and tertiary; depending on the time to progression from the moment the infection is acquired. Each stage involves various clinical manifestations; cardiovascular symptoms are part of tertiary syphilis, and syphilitic aortitis is the main form of presentation. We present the case of a patient who consulted with a focal neurological deficit, in whom extension studies reported neurosyphilis and severe aortic failure secondary to perforation of the right coronary valve, which required aortic valve replacement by bioprosthesis. We also undertake a review of the main cardiovascular manifestations of this disease. Although in the post-antibiotic era this type of late manifestation is increasingly less frequent, it is imperative that we are aware of it.

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.

Immunoglobulin G4-Related Aortitis of the Abdominal Aorta / 대한흉부외과학회지

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

Aortitis sifilítica: "una vieja olvidada" / Syphilitic aortitis: Old and forgotten. Report of a case

Resumen Se expone el caso de un paciente de sexo masculino, de 69 años, con antecedentes de hipertensión arterial, tabaquismo, ataque cerebrovascular, fibrilación auricular, insuficiencia aórtica y cardiopatía isquémica, quien ingresa a un hospital de alta complejidad por deterioro de la clase funcional, edema de miembros inferiores y episodios de palpitaciones. En la ecocardiografía se halló fracción de eyección normal, insuficiencia aórtica grave y dilatación de aorta ascendente con criterios quirúrgicos. Dentro de los estudios prequirúrgicos, se documentó VDRL reactivo en títulos altos y confirmación del diagnóstico de sífilis con TP-PA. Se realizó reemplazo valvular aórtico con prótesis biológica e injerto de dacrón en aorta ascendente, y el estudio patológico del material quirúrgico confirmó aortitis sifilítica. Se presenta una revisión respecto a esta patología poco frecuente en la actualidad.

Abstract The case is presented of a 69-year-old male patient with a history of arterial hypertension, smoking, stroke, atrial fibrillation, aortic regurgitation, and ischaemic heart disease, who was admitted to a tertiary hospital due to functional class deterioration, lower limb oedema, and episodes of palpitations. A normal ejection fraction, with severe aortic regurgitation and ascending aortic dilation with surgical criteria, was found on echocardiography. Within pre-surgical studies, the VDRL had high titres and confirmation of the diagnosis of syphilis with TP-PA. Aortic valve replacement was performed using a biological prosthesis and Dacron graft in the ascending aorta. The histopathology study of the surgical material confirmed syphilitic aortitis. A review is presented on the current status of this rare pathology.

Role of PET/CT in the Evaluation of Aortic Disease / 전남의대학술지

Positron emission tomography (PET) /computed tomography (CT) has been established as a standard imaging modality in the evaluation of malignancy. Although PET/CT has played a major role in the management of oncology patients, its clinical use has also increased for various disorders other than malignancy. Growing evidence shows that PET/CT images have many advantages in aortic disease as well. This review article addresses the potential role of PET/CT in diseases involving the aorta, emphasizing its usefulness with regard to acute thoracic aortic syndromes, aortic aneurysm, atherosclerotic lesions, aortitis and aortic tumors.

Arteritis de células gigantes relacionada a grandes vasos: a propósito de un caso / Large vessel giant cell arteritis: a case report

La arteritis de células gigantes corresponde a una vasculitis granulomatosa que afecta arterias de mediano y gran tamaño. El fenotipo clínico más conocido es la arteritis de la temporal, caracterizado por síntomas craneales clásicos como cefalea temporal, claudicación mandibular y síntomas visuales. Sin embargo, esta enfermedad puede comprometer otras grandes arterias como la aorta y sus ramas principales, denomi-nándose así, como arteritis de células gigantes de grandes vasos, la cual puede o no estar asociada a síntomas craneales. Presentamos el caso de una mujer de 74 años, con un cuadro de un mes de evolución, caracterizado por claudicación intermitente de extremidades inferiores, asociado a baja de peso de 3 kilos, sudoración nocturna. Al examen físico, fiebre y pulsos dismi-nuidos en extremidades inferiores.

Giant cell arteritis is a granulomatous vasculitis that affects arteries of medi-um and large size. The most well-known clinical phenotype is temporal arteri-tis, characterized by classic cranial symptoms such as temporal headache, man-dibular claudication and visual symptoms. However, this disease can involve other large arteries such as the aorta and its main branches, known as large ves-sel giant cell arteritis, which may or may not be associated with cranial symptoms.A 74-year-old woman is presented with claudication of lower extremities, associated with weight loss of 3 kilos, night sweats and fever over the past month. Physical ex-amination reveals decreased pulses in the lower extremities.

Cogan's syndrome - A rare aortitis, difficult to diagnose but with therapeutic potential / Síndrome de Cogan - Uma aortite rara, de diagnóstico difícil, mas com potencial terapêutico

Summary The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.

Resumo A inflamação da parede da aorta, denominada aortite, é uma condição clínica rara, que pode ser causada por diversas patologias, principalmente as de fundo inflamatório e/ou infeccioso. Nesse contexto, a ocorrência de sintomas vestibulares e oftalmológicos associados ao quadro remete ao diagnóstico de síndrome de Cogan, tornando o caso raro, mas passível de tratamento imunossupressor adequado e controle satisfatório da doença.

Aneurisma aórtico sifilítico en la era antibiótica, una vieja enfermedad con nueva historia / Syphilitic aortic aneurysm in the antibiotic era, an old disease with a new history

Los aneurismas de la aorta abdominal se presentan en el 5% a 10% de hombres entre 65 a 79 años de edad; es una patología multicausal que finalmente destruye la túnica media y altera la conformación de las fibras de colágeno de la pared arterial. Entre las principales causas se cita la aterosclerosis, enfermedades hereditarias (como el síndrome de Marfán), patologías inflamatorias y con baja prevalencia a la acción de agentes infecciosos (Salmonella, estafilococo dorado, Mycobacterium tuberculosis y Treponema pallidum). Se presenta el caso de un paciente con aneurisma de origen sifilítico, considerado una rareza médica en países desarrollados. No existen reportes en el país sobre casos de sífilis terciaria cardiovascular. Se complementa con una revisión bibliográfica de la epidemiología, clínica, diagnóstico y manejo. (AU)

Abdominal aortic aneurysms occur in 5% to 10% of men between 65 and 79 years of age. It has a multicausal pathology that ultimately destroys the tunica media and alters the conformation of the collagen fibers of the arterial wall. The main causes include atherosclerosis, hereditary diseases (such as Marfan syndrome), inflammatory pathologies and low prevalence of infectious agents (Salmonella, staphylococcus, Mycobacterium tuberculosis and Treponema pallidum). We present the case of a patient with an aneurysm of syphilitic origin, considered a medical rarity in developed countries. There are no reports in the country on cases of cardiovascular tertiary syphilis. It is complemented with a scientific literature review of epidemiology, clinical diagnosis and management. (AU)

A Surgical Case of Right Coronary Ostial Stenosis, Aortic Regurgitation, and Annuloaortic Ectasia Associated with Syphilitic Aortitis / 日本心臓血管外科学会雑誌

<p>We report a case of syphilitic aortitis (SA) associated with severe right coronary ostial stenosis, aortic regurgitation (AR), and annuloaortic ectasia (AAE). A 48-year-old man presented to a regional hospital with easy fatigability and nocturnal dyspnea. Echocardiography revealed Seller's grade 3 AR. A computed tomography scan showed AAE, dilatation of the ascending aorta, and calcification of both coronary ostia. Coronary angiography demonstrated that the left coronary artery was intact ; however, the right coronary artery was obscure. Active syphilis was detected on routine blood tests on admission. Therefore, the patient was started on a course of ampicillin/sulbactam (ABPC/SBT). Subsequently, he underwent the Bentall procedure and coronary artery bypass grafting with the right internal thoracic artery. The intraoperative findings showed degeneration of the aorta and severe right coronary ostial stenosis. The pathological findings of the aortic wall and aortic valve were consistent with SA. The postoperative course was uneventful. The patient continued receiving ABPC/SBT for 3 weeks postoperatively, and was then switched to oral amoxicillin.</p>

Paquimeningitis, aortitis y nefritis simulando arteritis temporal / Pachymeningitis, aortitis and nephritis simulating temporal arteritis

Resumen Se presenta el caso de un hombre de 65 años con cuadro clínico de cefalea y velocidad de sedimentación globular elevada en quien se sospechó arteritis de células gigantes (ACG), pero durante el proceso diagnóstico se le documentó paquimeningitis, hiperproteinorraquia y biopsia de arteria temporal reportada como normal. La búsqueda de otras enfermedades sistémicas que explicaran el cuadro clínico evidenció además la presencia de aortitis, glomerulonefritis y anticuerpos anticitoplasma de neutrófilos (ANCA) positivos, lo cual permitió hacer el diagnóstico de granulomatosis con poliangeítis (GP). Se presenta el análisis y enfoque diagnóstico de esta inusual asociación de paquimeningitis, aortitis y glomerulonefritis. (Acta Med Colomb 2016; 40: 259-265).

Abstract The case of a 65-year-old man with a clinical picture of headache and elevated erythrocyte sedimentation rate in whom giant cell arteritis (GCA) was suspected, but that during the diagnostic process was documented as pachymeningitis, hyperproteinorrachia and temporal artery biopsy reported as normal, is presented. The search for other systemic diseases that could explain the clinical picture also revealed the presence of aortitis, glomerulonephritis and anti-neutrophil cytoplasmic antibodies (ANCA), and allowed to make the diagnosis of granulomatosis with poliangeítis (GPA). The analysis and diagnostic approach of this unusual association of pachymeningitis, aortitis and glomerulonephritis is reported. (Acta Med Colomb 2016; 40:259-265).