Acometimento vascular na doença de Behçet: o processo imunopatológico

Abstract

Resumo A doença de Behçet constitui uma forma rara de vasculite sistêmica, que acomete de pequenos a grandes vasos. É caracterizada por manifestações mucocutâneas, pulmonares, cardiovasculares, gastrointestinais e neurológicas. Sua apresentação clínica é bastante ampla, variando de casos mais brandos a casos graves, com acometimento multissistêmico, caracteristicamente com exacerbações e remissões. Suas causas ainda são desconhecidas; entretanto, há evidências genéticas, ambientais e imunológicas, como a associação com o alelo HLA-B51. Todas essas, em conjunto, apontam para um processo imunopatológico anormal, com ativação de células da imunidade inata e adaptativa, como as células natural killer, neutrófilos e células T, que geram padrões de respostas e citocinas específicos capazes de gerar mediadores que podem lesionar e inflamar o sistema vascular, resultando em oclusões venosas, arteriais e/ou formação de aneurismas.

Endovascular treatment in Behçet’s disease: an integrative review

“…29 However, data on the ideal therapeutic approaches are still scarce and there is a lack of informative laboratory markers to monitor disease progression. 10 Mycotic aneurysms have incidence of 1% of all aneurysms and are caused by remote bacterial infections. Intravascular grafts are contraindicated when treating these false aneurysms, but successful stenting via endovascular surgery is described in the literature and reduces the risks of conventional surgery, particularly in the thoracic aorta or involving multiple segments.…”

“…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%.…”

“…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. 10 As a result, BD is a clinical condition that is associated with serious complications and causes significant morbidity and mortality. 15 Epidemiologically, BD has greatest prevalence among young adults aged 20 to 40 years, with peak incidence at 30 years of age, and affects both females and males, but the condition is more severe in males.…”

“…However, data on these ideal therapeutic approaches are still scarce and there is a lack of informative laboratory biomarkers to monitor disease progression. 10 With regard to the disease's vascular manifestations, aneurysms are rare, but since aneurysm rupture is the principal cause of death from this disease, they should be treated as soon as possible after identification. 3 Aneurysms in BD patients are treated whenever possible, because of the high risk of rupture, 20 but this is the only situation in which anticoagulants should not be used.…”

Síndrome de Behçet – os desafios do diagnóstico

“…Apesar dos avanços terapêuticos, ainda há escassez de dados sobre essas abordagens terapêuticas ideais e falta de marcadores laboratoriais informativos para monitorar a progressão da doença (15).…”