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.

Tumor retroperitoneal en el espectro de enfermedades asociadas a igg4 / Retroperitoneal tumor in the clinical spectrum of igg4 related disease

Presentamos el caso de una mujer de 55 años sin antecedentes, que acude por dolor abdominal, se realiza una tomografía abdominopélvica evidenciando un tumor retroperitoneal de aspecto quístico en la periferia de la aorta abdominal, que afecta el riñón izquierdo. Se le realizó una laparotomía exploratoria, nefrectomía izquierda, tumorectomía retroperitoneal y una resección de un fragmento de la aorta abdominal con reporte histopatológico, corroborado por una prueba inmunohistoquímica de fibrosis retroperitoneal y aortitis por IgG4. El espectro de enfermedades relacionadas con IgG4 es de origen inmunológico con la capacidad de involucrar casi cualquier órgano. La epidemiología no es precisa, aunque se observa mayor predilección por la raza asiática, siendo más común durante la séptima década de vida. El diagnóstico radica en la coexistencia de varios parámetros clínicos, laboratoriales e histopatológicos, sin ser ninguno de estos patognomónicos. El objetivo del presente artículo es exponer el reporte de un caso con manifestación inusual de fibrosis retroperitoneal y dar a conocer que la importancia del diagnóstico radica en la decisión terapéutica, ya que cursa con buena respuesta al tratamiento inmunosupresor.

We present the case of a 55-year-old woman, with noclinical pathological history, who presented with abdominal pain, abdominopelvic tomography showing retroperitoneal tumoral in the periphery of the abdominal aorta, which affects the left kidney producing hydronephrosis. Exploratory laparotomy, left radical nephrectomy, retroperitoneal tumorectomy and resection of a fragment of abdominal aorta were performed, with histopathological report, corroborated by immunohistochemistry of retroperitoneal fibrosis and IgG4 aortitis. The spectrum of diseases related to IgG4, are of immunological origin that can involve almost any organ. The epidemiology is not precise, although a greater predilection for the Asian race is observed, being more common in the seventh decade of life, it should be suspected in patients with unexplained pain in one or more organs.The diagnosis lies in the coexistence of several clinical, laboratorial and histopathological parameters, without being any of these pathognomonic. We present a case with unusual presentation of retroperitoneal fibrosis and the importance of the diagnosis lies in the therapeutic decision, since it responds well to immunosuppressive treatment, without requiring surgical management.

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.

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.

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.

Cogan Syndrome Accompanied with Meningitis / 대한평형의학회지

Cogan syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly and diarrhea. We report a case of Cogan syndrome accompanied with meningitis.

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.

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).

Arteritis de células gigantes: compromiso extenso de la aorta y grandes ramas demostrado por tomografía por emisión de positrones. Caso clínico / Extensive arterial involvement in giant cell arteritis: report of one case

Large vessel vasculitis and particularly Temporal Arteritis are systemic diseases that may affect the aorta and its major branches, mainly involving extra cranial branches of the carotid artery. We report a 72-year-old man presenting with weight loss, fever and malaise. Positron emission computed tomography (PET CT) showed an extensive inflammation of the aorta and its major branches. Temporal artery biopsy confirmed the presence of vasculitis with granulomas. Treatment with a high dose of steroids had an excellent clinical response. This case underscores the utility of PET CT in the assessment of this disease.

A Rapidly Expanding Ascending Aortic Aneurysm in a Patient with Relapsing Polychondritis / 대한내과학회지

Here we describe a case of rapidly expanding ascending aortic aneurysm in a patient with relapsing polychondritis. To prevent aneurysm rupture, the patient underwent emergent surgical repair. Silent inflammation can progress in the aorta wall, even in asymptomatic patients with mild disease activity under immunosuppressive treatment, leading to the rapid growth of aortic aneurysms. Close monitoring with routine imaging is needed once a patient with relapsing polychondritis is diagnosed with an aortic aneurysm.

Long-Term Outcomes of Homografts in the Aortic Valve and Root Position: A 20-Year Experience

BACKGROUND: The advantages of using a homograft in valve replacement surgery are the excellent hemodynamic profile, low risk of thromboembolism, and low risk of prosthetic valve infection. The aim of this study was to evaluate the long-term outcomes of homograft implantation in the aortic valve position. METHODS: This is a retrospective study of 33 patients (>20 years old) who underwent aortic valve replacement or root replacement with homografts between April 1995 and May 2015. Valves were collected within 24 hours from explanted hearts of heart transplant recipients (<60 years) and organ donors who were not suitable for heart transplantation. The median follow-up duration was 35.6 months (range, 0 to 168 months). RESULTS: Aortic homografts were used in all patients. The 30-day mortality rate was 9.1%. The 1- and 5-year survival rates were 80.0%±7.3% and 60.8%±10.1%, respectively. The 1-, 5-, and 10-year freedom from reoperation rates were 92.3%±5.2%, 68.9%±10.2%, and 50.3%±13.6%, respectively. The 1-, 5-, and 10-year freedom from significant aortic dysfunction rates were 91.7%±8.0%, 41.7%±14.2%, and 25.0%±12.5%, respectively. CONCLUSION: Homografts had the advantages of a good hemodynamic profile and low risk of thromboembolic events, and with good outcomes in cases of aortitis.

Vasculitis de grandes vasos en paciente con vasculitis ANCA (+): Caso clínico / Large vessel involvement in ANCA-associated vasculitis: Report of one case

Pauci-immune glomerulonephritis in systemic vasculitides usually have anti-neuthrophil cytoplasmic antibodies (ANCA). However, vasculitides of large vessels such as Takayasu’s and giant cell (temporal) arteritis do not. Exceptionally ANCA(+) small vessel vasculitides are associated with large vessel vasculitis. It may be a coincidence or both vasculitides have a common pathogenesis. We report a 30 years old woman on hemodialysis due to a chronic glomerulonephritis ANCA(+) diagnosed nine years ago. Eight years later, she presented with an aortitis with severe stenosis of distal aorta and vasculitis of left subclavian artery. She was treated with adrenal steroids and cyclophosphamide. During the ensuing five years she has been stable and without signs of reactivation of the disease.

Aortic thrombosis in a patient with a late of Behcet's disease / Trombose aórtica em paciente com diagnóstico tardio de doença de Behçet

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.

A Doença de Behçet é uma vasculite sistêmica de etiologia desconhecida. O inusitado é que o acometimento arterial nesta doença é menos comum que o venoso, correspondendo a 1 a 7% dos pacientes. Já os sintomas vasculares precedem o diagnóstico clínico de Behçet em 7 a 30%. Relatamos o caso de um paciente portador de Tromboangeíte Obliterante que foi submetido a simpatectomia lombar e derivação aorto-bi-ilíaca. Após cerca de um ano, diagnosticou-se Doença de Behçet, devido a quadro clínico de úlceras orais, genitais e lesões descamativas nas mãos.

A Case of Aortitis with Systemic Lupus Erythematosus

A 21-year-old woman with a history of systemic lupus erythematosus (SLE) was admitted with dyspnea on exertion for a year. A transesophageal echocardiogram showed dilated aortic root with intimal thickening. A positron emission tomography/computed tomography demonstrated increase in glucose hypermetabolic along the walls of the aortic valve, ascending aorta, aortic arch, and aorta, vasculitis was observed. She underwent the Bentall operation due to inflammation at sinus of right coronary cusp. She started high dose glucocorticoid after the operation. Currently she is able to sustain with low dose steroid after gradually tapered. Her symptoms were disappeared, and inflammatory markers decreased to within the normal range. Aortitis and aortic aneurysms are an uncommon manifestation of SLE. Furthermore, almost of lupus patients with medium and large vessel vasculitis are not biopsied or studied histologically. We present first case in Korea that was a 21-year-old woman who diagnosed with lupus aortitis by pathology after aortic valve replacement operation.

Abdominal aortic aneurysm in giant cell arteritis

Aortic complications of giant cell arteritis are a rare cause of abdominal aortic aneurysm. Here, we describe a case of a ruptured aortic aneurysm in a patient with giant call arteritis (GCA) who was preoperatively suspected of having an infectious aortic aneurysm. Intraoperative inspection revealed infectious granulation tissue on the anterior wall of the abdominal aorta. GCA was finally confirmed by pathological diagnosis. Our findings suggest that the surgical and postoperative treatment of nonatheromatous aortic aneurysm should be based on accurate diagnosis.