Correção endovascular de pseudoaneurisma toracoabdominal em paciente com Doença de Behçet

Abstract

Behçet' s disease is an inflammatory disease characterized by recurrent oral and genital ulcers, uveitis and skin lesions. Arterial involvement is rare, but when present aneurysmal degeneration is more common than occlusive disease. This report describes the clinical case of a female patient who had been receiving treatment for Behçet' s disease for twenty years before presenting with abdominal pain that progressed for 2 months before suddenly worsening significantly. A CT scan revealed a thoracoabdominal pseudoaneurysm. She was successfully treated with endovascular repair.Keywords: Behçet' s disease; aortic aneurysm; dissecting aneurysm; endovascular procedures. ResumoA Doença de Behçet é uma doença inflamatória caracterizada por úlceras genitais e orais recorrentes, uveítes e lesões cutâneas. O envolvimento arterial é raro, sendo mais comuns as degenerações aneurismáticas do que as oclusivas. Neste caso clínico, paciente do sexo feminino, em tratamento de doença de Behçet havia 20 anos, iniciou com dor abdominal progressiva por dois meses, com piora súbita importante, foi submetida à tomografia computadorizada, que mostrou pseudoaneurisma toracoabdominal. A paciente foi submetida a tratamento endovascular com sucesso.Palavras-chave: Doença de Behçet; aneurisma aórtico; aneurisma dissecante; procedimentos endovasculares.

Tratamento endovascular da doença de Behçet: relato de caso

“…10,13 In the literature, rates of vascular involvement are variable, affecting from 7 to 29% of patients with BD. 2,4,8,11,15,16 The disease can involve both the venous system and the arterial system, causing thromboses venous/thrombophlebitis and varicose veins in around 88% of patients with vascular injuries. Arterial involvement, including occlusions/stenoses, aneurysms, and pseudoaneurysms, are responsible for approximately 12% of cases.…”

“…Arterial involvement, including occlusions/stenoses, aneurysms, and pseudoaneurysms, are responsible for approximately 12% of cases. [1][2][3][4]6,7,12,16,17 Although less frequent, arterial injuries, such as aneurysms, are the major cause of death. 2,15,18 The abdominal aorta is the most commonly affected site, followed by the femoral and pulmonary arteries.…”

“…2,15,18 The abdominal aorta is the most commonly affected site, followed by the femoral and pulmonary arteries. 3,4,[6][7][8]10,18 The origin of BD it is not yet clear, 2,7,15 but it is believed that an autoimmune phenomenon takes place and triggers an inflammatory process. This causes degeneration of the vasa vasorum, thickening of the tunica intima, and destruction of the elastic fibers of the walls of vessels.…”

“…In some cases, immunosuppressants are used to provoke a remission of the disease, reducing inflammation of the vessels and the risk of postoperative complications. 2,3,4,6,7,[12][13][14][15]17 It is known that aneurysms caused by BD exhibit a vigorous inflammatory process in the artery walls, expand rapidly, and have an elevated risk of rupture that is not correlated with their size and, because of this, must be treated whenever possible. 10,11 Treatment of the vascular injuries seen in these patients is a major challenge for vascular surgeons, because of the technical difficulties involved and the high rates of complications, such as formation of pseudoaneurysms at anastomosis sites, which occur in 30 to 50% of patients with BD.…”

“…2 Diagnosis is essentially clinical and there is no specific diagnostic test. 3,4 There is an international criterion for diagnosis/classification of BD, based on a scoring system that attributes two points each to ocular lesions and genital ulcers and one point to each of the following items: oral apthous ulcers; cutaneous manifestations; vascular manifestations; and a positive pathergy test. Three or more points are needed to define a patient as a case of BD.…”