Tratamento endovascular de aneurisma sacular aórtico associado à doença de Adamantiades-Behçet

Abstract

Resumo A doença de Adamantiades-Behçet é uma desordem multissistêmica que se apresenta classicamente com úlceras orais e genitais e envolvimento ocular, podendo o acometimento vascular ocorrer em até 38% dos casos. O envolvimento aórtico é uma das manifestações mais severas e está associado a altas taxas de mortalidade, ocorrendo em 1,5 a 2,7% dos casos. Relatamos um caso de aneurisma sacular de aorta abdominal em um paciente de 49 anos com doença de Adamantiades-Behçet complicada, tratada por correção endovascular.

Endovascular treatment in Behçet’s disease: an integrative review

“…1 However, many authors interested in the disease prefer the term "Behçet syndrome" to "Behçet's disease", because its manifestations and severity can vary considerably between patients and even in terms of the prevalence of individual manifestations in different parts of the world, in particular those related to intestinal involvement. 2 Behçet's disease is rare, chronic, relapsing, inflammatory, and multisystemic 3,4 and can provoke mucocutaneous, ocular, vascular, cardiac, neural, and gastrointestinal manifestations. 5 The international diagnostic criteria for BD are recurrent oral ulceration (apthous ulcerations that recur three or more times in a 12-month period) and at least two of the following: recurrent genital ulceration (apthous ulcer or scabs), ocular lesions (anterior uveitis or retinal vasculitis), cutaneous lesions (erythema nodosum, pseudofolliculitis, or papulopustular lesions), and pathergy (skin hyperreactivity test in response to a minimal trauma, with readings in 24-48 hours) 6,7 Vascular involvement is one of the primary predictors of morbidity and mortality in BD, with a negative impact on prognosis.…”

“…9 The vascular form of the disease, vasculo-Behçet, is present in around 7 to 38% of cases and venous involvement is more common than arterial. 3,10 Arterial injuries have prevalence ranging from 3.6 to 31%, 3 generally develop in the aorta and pulmonary artery and their main branches, 11 and can vary from aneurysms to acute arterial thromboses or stenoses, most often affecting the renal, pulmonary, and abdominal arteries. 3 An aneurysm tends to be present in 65% of patients with arterial injury and occlusion is found in 35%.…”

“…3,10 Arterial injuries have prevalence ranging from 3.6 to 31%, 3 generally develop in the aorta and pulmonary artery and their main branches, 11 and can vary from aneurysms to acute arterial thromboses or stenoses, most often affecting the renal, pulmonary, and abdominal arteries. 3 An aneurysm tends to be present in 65% of patients with arterial injury and occlusion is found in 35%. 11 The most common manifestation is deep venous thrombosis in lower limbs.…”

“…Aortic involvement is one of the most serious manifestations and is associated with high mortality rates. 3 The mechanism of thrombosis in BD is not yet known, although antiphospholipid antibodies have been reported in some studies, but their association with BD is unclear. 12 Occlusive arterial disease and formation of aneurysms both occur as manifestations of BD, but arterial aneurysms occur with greater frequency than occlusive disease.…”

“…Arterial disease generally manifests from 3 to 8 years after the initial diagnosis, with mean age at onset of 30 years. 13 The pathophysiology of BD has not yet been clearly defined, although it is known to be autoimmune in nature, 14 involving the antigens HLA-B51 and HLA-B27 3 and interaction between genetic and environmental factors, such as infection by bacteria of the genus Streptococcus, 14 infection by the herpes simplex virus, and family history. It is thus believed that there is an abnormal immunopathological process capable of injuring and inflaming the vascular system, leading to occlusions of the circulation and to aneurysms.…”