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.

[Aortitis: report of three cases]. / Aortitis, causas infrecuentes en Reumatología: presentación de casos.

Aortitis is a nonspecific term that describes an inflammation of the aortic wall caused by inflammatory, infectious, paraneoplastic and idiopathic diseases. The symptoms are variable and nonspecific; therefore a high level of clinical suspicion is required to diagnose it. It is often an incidental finding while looking for other diagnoses and it is confirmed mainly through imaging studies. We report three cases of aortitis: A 29-year-old woman presenting with alopecia, oral and nasal ulcers and positive antinuclear antibodies. A CAT scan showed a segmental thickening of thoracic aorta, with dilated and stenotic areas. She was successfully treated with steroids, hydroxychloroquine, cyclophosphamide and azathioprine. A 41-year-old male presenting with dorsal pain and cough. The CAT scan showed an extra-intimal thickening of the descending aorta and stenosis of the celiac artery. The final diagnosis was a polyangiitis and was treated with steroids, cyclophosphamide and azathioprine. A 28-year-old woman presenting with pain in the left upper abdomen. Imaging studies showed a thickening of the aortic arch and subclavian artery. The final diagnosis was sarcoidosis and the patient was treated with prednisone.

Aortitis, causas infrecuentes en Reumatología: presentación de casos / Aortitis: Report of three cases

Aortitis is a nonspecific term that describes an inflammation of the aortic wall caused by inflammatory, infectious, paraneoplastic and idiopathic diseases. The symptoms are variable and nonspecific; therefore a high level of clinical suspicion is required to diagnose it. It is often an incidental finding while looking for other diagnoses and it is confirmed mainly through imaging studies. We report three cases of aortitis: A 29-year-old woman presenting with alopecia, oral and nasal ulcers and positive antinuclear antibodies. A CAT scan showed a segmental thickening of thoracic aorta, with dilated and stenotic areas. She was successfully treated with steroids, hydroxychloroquine, cyclophosphamide and azathioprine. A 41-year-old male presenting with dorsal pain and cough. The CAT scan showed an extra-intimal thickening of the descending aorta and stenosis of the celiac artery. The final diagnosis was a polyangiitis and was treated with steroids, cyclophosphamide and azathioprine. A 28-year-old woman presenting with pain in the left upper abdomen. Imaging studies showed a thickening of the aortic arch and subclavian artery. The final diagnosis was sarcoidosis and the patient was treated with prednisone.

Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases.

OBJECTIVE: To determine 1) the frequency of idiopathic aortitis in a large surgical cohort, 2) how often aortitis was associated with a systemic disease, and 3) whether the findings of aortitis in resected specimens predicted future occurrences of clinically apparent vascular injury due to vasculitis. METHODS: Retrospective chart and pathology review of 1,204 aortic surgical specimens that were gathered over a period of 20 years at a tertiary care medical center. A standardized database was used to compare features of aortitis patients with those of controls in whom inflammation was not present. RESULTS: Among 1,204 aortic specimens, 52 (4.3%) were clinically and pathologically classified as idiopathic aortitis. Sixty-seven percent of patients with idiopathic aortitis were women. In 96% of idiopathic aortitis patients with aneurysm formation, aortitis was present only in the thoracic aorta. Among 383 thoracic aortic aneurysms, 12% had idiopathic inflammatory features. In 96% of patients with idiopathic aortitis, symptoms of systemic illness had not been present at the time of surgery. In 31%, aortitis was associated with a remote history of vasculitis and a variety of other systemic disorders. During a mean followup period of 41.2 months, new aneurysms were identified among 6 of 25 patients who were not treated with glucocorticoids. None were identified among 11 patients who were treated with glucocorticoids (mean followup 35.5 months). CONCLUSION: The frequency of idiopathic aortitis in a large surgical cohort was found to be 4.3%. Thoracic aorta aneurysm formation, in the absence of systemic illness, was the most common manifestation. In the setting of a cardiovascular surgery practice, aortitis may first become apparent only after pathologic evaluation of excised specimens. The appropriate medical treatment for patients with incidentally discovered aortitis is not known. Because 17% of our patients subsequently developed new aneurysms, we suggest that it would be prudent for patients with idiopathic aortitis identified at the time of surgery to be periodically evaluated for recurrent or persistent disease.

-Prolonged fever syndrome and infection of abdominal aortic aneurysm due to Salmonella enteritidis. / Síndrome febril prolongado e infección de aneurisma de aorta abdominal por Salmonella enteritidis.

Endovascular infection of atherosclerotic aorta is a rare event in the setting of aged patients with gram negative bacteremia of the salmonella group. Until the beginning of the 60s this meant an ominous diagnosis with an almost unavoidable fatal prognosis. Presently, this trend has been reverted, mostly due to an earlier diagnosis, the development of more sophisticated imaging techniques, the correct use of broad spectrum bactericidal antibiotics and prompt surgical management. Paradoxically, the incidence of arterial infections has increased in recent years, specially in old people with atherosclerotic abdominal aortic aneurysms, in whom infective endocarditis could not be demonstrated. We describe the case of a 65 year old man, with a history of longstanding non-insulin-dependent diabetes, presenting with protracted fever, weight loss and thigh pain. Blood cultures and serologic studies as well as several echocardiograms yielded negative results. An abdominal CT scan showed an infrarenal aortic aneurysm raising the clinical suspicion of arterial infection of abdominal aorta. The patient underwent surgery because of highly presumptive diagnosis of complicated aortic aneurysm. The resection was followed by an in situ graft. There was no evidence of disruption or gross collection. Samples of the aortic wall and perianeurysmatic fluid grew Salmonella enteritides. We describe the main etiopathogenic and clinic features of the entity highlighting the high sensitivity and specificity of the CT scan in the identification and characterization of infected aortic aneurysm. Certain features may firmly suggest this diagnosis without using preoperative aortography.

Síndrome febril prolongado e infección de aneurisma de aorta abdominal por Salmonella enteritidis / Prolonged fever syndrome and infection of abdominal aortic aneurism due to Salmonella enteritidis

Endovascular infection of atherosclerotic aorta is a rare event in the setting of aged patients gram negative bacteremia of the salmonella group. Until the beginning of the 60s this meant an ominous diagnosis with an almost unavoidable fatal prognosis. Presently, this trend has been reverted, mostly due to an earlier diagnosis, the development of more sophisticated imaging techniques, the correct use of broad spectrum bactericidal antibiotics and prompt surgical management. Paradoxically, the incidence of arterial infections has increased in recent years, specially in old people with atherosclerotic abdominal aortic aneurysms, in whom infective endocarditis could not be demonstrated. We describe the case of a 65 year old man, with a history of long-standing non-insulin-dependent diabetes, presenting with protracted fever, weight loss and thigh pain. Blood cultures and serologic studies as well as several echocardiograms yielded negative results. An abdominal CT scan showed an infrarenal aortic aneurysm raising the clinical suspicion of arterial infection of abdominal aorta. The patient underwent surgery because of highly presumptive diagnosis of complicated aortic aneurysm. The resection was followed by an in situ graft. There was no evidence of disruption or gross collection. Samples of the aortic wall and perianeurysmatic fluid grew Salmonella enteritides. We describe the main etiopathogenic and clinic features of the entity highlighting the high sensitivity and specificity of the CT scan in the identification and characterization of infected aortic aneurysm. Certain features may firmly suggest this diagnosis without using preoperative aortography.

Síndrome febril prolongado e infección de aneurisma de aorta abdominal por Salmonella enteritidis / Prolonged fever syndrome and infection of abdominal aortic aneurism due to Salmonella enteritidis

Endovascular infection of atherosclerotic aorta is a rare event in the setting of aged patients gram negative bacteremia of the salmonella group. Until the beginning of the 60s this meant an ominous diagnosis with an almost unavoidable fatal prognosis. Presently, this trend has been reverted, mostly due to an earlier diagnosis, the development of more sophisticated imaging techniques, the correct use of broad spectrum bactericidal antibiotics and prompt surgical management. Paradoxically, the incidence of arterial infections has increased in recent years, specially in old people with atherosclerotic abdominal aortic aneurysms, in whom infective endocarditis could not be demonstrated. We describe the case of a 65 year old man, with a history of long-standing non-insulin-dependent diabetes, presenting with protracted fever, weight loss and thigh pain. Blood cultures and serologic studies as well as several echocardiograms yielded negative results. An abdominal CT scan showed an infrarenal aortic aneurysm raising the clinical suspicion of arterial infection of abdominal aorta. The patient underwent surgery because of highly presumptive diagnosis of complicated aortic aneurysm. The resection was followed by an in situ graft. There was no evidence of disruption or gross collection. Samples of the aortic wall and perianeurysmatic fluid grew Salmonella enteritides. We describe the main etiopathogenic and clinic features of the entity highlighting the high sensitivity and specificity of the CT scan in the identification and characterization of infected aortic aneurysm. Certain features may firmly suggest this diagnosis without using preoperative aortography.(AU)