Tratamento endovascular de aneurisma de aorta abdominal com erosão de vértebra lombar associada à doença de Behçet: relato de caso

Abstract

Behçet' s disease is an autoimmune, multifactorial, systemic condition with several clinical manifestations, including vascular disorders. An aortic aneurysm with vertebral erosion is rare in association with this pathology and there are only four case reports listed on the PubMed database. This article reports the case of a female patient with a long-standing diagnosis of Behçet' s Disease who developed a saccular infrarenal abdominal aortic aneurysm with lumbar vertebral erosion. Her surgical treatment consisted of endovascular repair with a monoiliac endoprosthesis and a femorofemoral crossover bypass, because of limitations imposed by the anatomy of the aortic bifurcation. This paper discusses the rarity of this presentation of the disease and treatment outcomes and offers a brief review of the relevant literature.Keywords: Behçet' s Disease; aneurysm; spinal column; vertebral lesions. ResumoA doença de Behçet é uma doença sistêmica, multifatorial e autoimune com diversas manifestações clínicas, entre elas o acometimento vascular. Aneurisma de aorta associado a erosão de vértebra lombar é condição rara na literatura, existindo apenas quatro relatos de caso nas bases de dados da PubMed. O presente artigo relata o caso de paciente do sexo feminino com diagnóstico de Doença de Behçet de longa data e aneurisma sacular de aorta abdominal infrarrenal com erosão de vértebra lombar. O caso foi tratado por meio de técnica endovascular com colocação de endoprótese monoilíaca e enxerto fêmoro-femoral cruzado, devido a limitações anatômicas da bifurcação aórtica. O artigo aborda a raridade desse tipo de apresentação da doença e o desfecho do tratamento e apresenta revisão da literatura sobre esse tema.Palavras-chave: Doença de Behçet; aneurisma; coluna vertebral; lesões de coluna vertebral.

Endovascular treatment in Behçet’s disease: an integrative review

“…9 Clinical treatment using corticosteroids and immunosuppressants (cyclophosphamide and azathioprine) 3 should be attempted before any surgical treatment, whether open or endovascular, since there is a high rate of aneurysmal disease recurrence among BD patients. 14 However, urgent treatment is indicated if remission of important symptoms does not occur and the risk of rupture does not recede with clinical treatment alone. 25 There is also the possibility of adjuvant use of corticosteroids or immunosuppressants during the postoperative period and also of use of anticoagulants or platelet antiaggregants, with the objective of reducing the risk of graft occlusion.…”

“…1 It is linked with a lower likelihood of complications during the postoperative period, with incidence of around 19%. 23 Endovascular treatment is therefore proving to be a promising alternative to open surgery for treating these patients 14 and has already become the approach of choice for the majority of patients with high surgical risk. 23 Endovascular treatment generally involves shorter operating time, shorter hospital stay, and lower blood loss compared with open surgical treatment.…”

“…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.…”

Tratamento endovascular na doença de Behçet: uma revisão integrativa

“…Clinical treatment using corticosteroids and immunosuppressants (cyclophosphamide and azathioprine) 3 should be attempted before any surgical treatment, whether open or endovascular, since there is a high rate of aneurysmal disease recurrence among BD patients. 14 However, urgent treatment is indicated if remission of important symptoms does not occur and the risk of rupture does not recede with clinical treatment alone. 25 There is also the possibility of adjuvant use of corticosteroids or immunosuppressants during the postoperative period and also of use of anticoagulants or platelet antiaggregants, with the objective of reducing the risk of graft occlusion.…”

“…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.…”