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.

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.

Inflammatory aortic aneurysm: a case report and review of literatures.

A case of an uncommon variant of aortic aneurysm, inflammatory type, is reported. A 51-year-old Thai male presented with a pulsatile abdominal mass associated with pain. Ultrasonography demonstrated infrarenal abdominal aortic aneurysm preoperatively and operative findings revealed dense fibrous tissue around the lesion. Serological tests for syphilis and bacteriological studies of aneurysm contents were all negative. Aneurysmorrhaphy was done, using Dacron straight graft, and two serious complications developed at six and two months interval: aortocolonic and aortoduodenal fistulae. However, the patient survived the three operations. Definite diagnosis of inflammatory aortic aneurysm was confirmed by typical pathological findings. Clinical presentations, operative and pathological findings were compared to previous literature.