Kaieda Shinjiro, Zaizen Yoshiaki, Nomura Yoriko, Okabe Koki, Honda Seiyo, Kage Masayoshi, Ida Hiroaki, Hoshino Tomoaki, Fukuda Takaaki
Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine , Kurume , Japan.
Mod Rheumatol. 2015 Mar;25(2):307-11. doi: 10.3109/14397595.2013.874755. Epub 2014 Feb 18.
Pulmonary vascular involvement in Behçet's disease is a rare complication with a poor prognosis. We present an autopsy case of vasculo-Behçet's disease complicated by pulmonary hemorrhage, possibly caused by rupture of pulmonary artery aneurysms. The patient was treated with a combination of high-dose steroids and pulse cyclophosphamide, but he died from massive hemoptysis. This case highlights the need for potent new therapies for patients with vasculo-Behçet's disease refractory to conventional immunosuppressive therapy, such as a combination of steroids and cyclophosphamide.
白塞病的肺血管受累是一种罕见的并发症，预后较差。我们报告一例血管型白塞病尸检病例，该病例并发肺出血，可能由肺动脉瘤破裂所致。患者接受了大剂量类固醇和脉冲环磷酰胺联合治疗，但死于大量咯血。该病例凸显了对于传统免疫抑制治疗（如类固醇和环磷酰胺联合治疗）难治的血管型白塞病患者，需要有效的新治疗方法。
