Barberis M, Casadio C, Borghini U
Istituto di Anatomia Patologica, Ospedale Niguarda-Cà Granda, Milano, Italia.
Respiration. 1987;52(4):303-7. doi: 10.1159/000195339.
Behçet syndrome in association with pulmonary manifestations is rare. We describe a patient suffering from recurrent oral and genital ulcerations, conjunctivitis, thrombophlebitis and fluctuating radiological opacities in the lungs who died after massive haemoptysis. The autopsy showed a necrotizing vasculitis involving pulmonary arteries, muscular arteries and veins. It was complicated by arterial thromboses, bronchial erosions by pulmonary artery aneurysms and formation of a large arteriobronchial fistula. We conclude that the spread of the inflammatory infiltrate from the wall of the vessels to the adjacent bronchi is responsible for these complications and that haemoptysis must be considered as the expression of dramatic progression of the disease.
白塞病合并肺部表现较为罕见。我们描述了一名患者，其反复出现口腔和生殖器溃疡、结膜炎、血栓性静脉炎，肺部影像学表现为多变的不透明影，最终因大量咯血死亡。尸检显示为坏死性血管炎，累及肺动脉、肌性动脉和静脉。并发症包括动脉血栓形成、肺动脉瘤导致的支气管侵蚀以及巨大动支气管瘘的形成。我们得出结论，炎症浸润从血管壁蔓延至相邻支气管是这些并发症的原因，咯血必须被视为疾病急剧进展的表现。
