貝西氏症併肺動脈瘤與咳血：二病例報告

Behcet's Disease Complicated with Pulmonary Arterial Aneurysms and Hemoptysis-- A Report of Two Cases

Behcet於1937年將臨床上的三種症狀－－葡萄膜炎、口腔及生殖器潰瘍，界定為一種徵候群，今此徵候群已知為一可侵犯各種器官的全身疾病。約百分之五至百分之十的病人併有肺部症狀，肺部症狀被認為是因肺血管炎所引起。肺動脈瘤是貝西氏症一個罕見但嚴重的併發症，在此 我們報告兩例貝西氏症併肺動脈瘤引起咳血的經驗。首例病人為二十三歲男性，有覆發性口腔潰瘍、兩側下肢結節性紅 與深部靜脈栓塞的病史。因反覆咳血一個多月住院，胸部電腦斷層及動脈血管攝影證實兩側肺脈瘤的存在。第二例為二十二歲女性貝西氏症病人，有復發性口腔及生殖器潰湯、結節性紅斑、關節炎與葡萄膜炎的病史。反覆咳血六個多月，胸部電腦斷層發現右下肺有一小的肺動脈瘤。此二例病人經秋水仙素及口服類固醇與Cyclophosphamide的治療，咳血及肺動脈瘤均呈緩解現象。合併療法具有良好的短期療效，長期療效則有待進一步的追蹤與評估。

The clinical triad of uveitis with oral and genital ulceration was first defined by Behcet in 1937 as a syndrome, but it is now recognized as a systemic disease involving various organs. Lung symptoms occur in 5 to 10% of patients and are thought to be caused by pulmonary vasculitis. Pulmonary arterial aneurysms are a rare, but serious, complication of Behcet's disease. This report concerns experience here with two patients with Behcet's disease, presenting with recurrent hemoptysis proved to result from pulmonary arterial aneurysms. The first case was a 23-year-old male admitted for recurrent hemoptysis occurring for more than one month. A history of recurrent oral ulcers, erythema nodosum and deep vein thrombosis were noted. Chest radiograph revealed bilateral hilar opacities. Later CT scan of chest and pulmonary arterial angiography confirmed bilateral pulmonary arterial aneurysms. The second case, a 22-year-old female of Behcet's disease, had oral and genital ulcers, erythema nodosum, arthritis and uveitis. Recurrent episodes of hemoptysis had occurred for more than 6 months, and CT scan of chest showed a small pulmonary arterial aneurysm over the right lower lung. Resolution of hemoptysis and radiologic signs of pulmonary arterial aneurysms were observed after colchicine, oral corticosteroid and cyclophosphamide treatment. Combination therapy consisting of colchicine, corticosteroid and cyclophosphamide had good results in the short term. However, long-term results need further follow-up and evaluation.