Behçet's disease with major vascular involvement

Abstract

A 40-year-old Chinese man was admitted for haemoptysis and progressive deep vein thrombosis involving the inferior vena cava (IVC) despite anticoagulation. An IVC filter had been placed earlier at an outside hospital. CT angiography revealed two pulmonary artery aneurysms. The patient was found to have a history of oral and genital ulcers, uveitis and erythema nodosum, thus meeting criteria for Behçet's disease. Other causes of the haemoptysis and thrombophilia were excluded. He underwent successful coil embolisation of the pulmonary artery aneurysms and responded well to immunosuppressive therapy with cyclophosphamide and steroids. Anticoagulation was cautiously continued to limit the long-term risk of secondary thrombosis from his IVC filter. The patient remains well 5 months after initiation of immunosuppressive therapy. Making a diagnosis of Behçet's disease in the setting of thrombosis is crucial, as treatment must include immunosuppression, and, thus, fundamentally differs from the management of most other thrombotic disorders.

Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

“…[70][71][72] There is a case report of a patient with BD, DVT and pulmonary artery aneurysm treated with inferior vena cava (IVC) filter, coil embolization for pulmonary artery aneurysm, anticoagulation and immunosuppression. 73 The data for the use of infra vena cava (IVC) filter in patients with BD are very limited. In a retrospective study from Turkey, one patient out of 52 patients with BD and venous thrombosis had IVC filter.…”

Hemoptisis como manifestación inicial de la enfermedad de Behçet. Casos clínicos

“…La enfermedad de Behçet es una vasculitis con afectación de múltiples órganos. El compromiso vascular, arterial o venoso, varía entre 11-30% de los casos [2][3][4]9,[13][14][15][16] . El compromiso venoso es más frecuente que el arterial, siendo la trombosis de extremidades inferiores la manifestación más habitual.…”

“…El compromiso venoso es más frecuente que el arterial, siendo la trombosis de extremidades inferiores la manifestación más habitual. Las embolías pulmonares son poco frecuentes, debido a que el trombo se encuentra fuertemente adherido a las paredes vasculares inflamadas [2][3][4]7,[13][14][15][16] .…”

“…Los aneurismas pueden complicarse con rupturas hacia el parénquima pulmonar, manifestándose clínicamente por hemoptisis. Esta es la principal causa de muerte de los pacientes con EB [2][3][4]7,[13][14][15][16][17][18][19] . En la literatura nacional sólo se han comunicado tres casos de compromiso pulmonar, con dos desenlaces fatales por hemoptisis masiva [9][10][11] .…”

“…Otros órganos afectados son las articulaciones, el tracto gastrointestinal y los pulmones 1,2,4,5,7,12 . El compromiso a nivel pulmonar es infrecuente, pero la ruptura de los aneurismas de las arterias pulmonares corresponde a la principal causa de muerte de esta enfermedad [2][3][4][9][10][11][13][14][15][16] .…”