白塞氏病合并肺动脉瘤附七例临床分析

目的探讨白塞氏病合并肺血管病变的特点,以提高对该病的早期认识和治疗水平。方法回顾性分析了1999年～2004年收住北京协和医院的白塞氏病合并肺血管病变的7例患者的临床、影像学及病理学资料。结果不同程度的咯血(7/7)是白塞氏病合并肺血管病的典型特点;影像学表现为肺门影增大及肺外周带大小不等、边缘整齐的结节影(7/7),肺血管造影显示相应部位肺动脉瘤形成(5/5)及远端血管闭塞。1例患者行肺叶切除术,病理学表现为血管的慢性炎症。6例患者经泼尼松和环磷酰胺联合治疗预后好,其中2例接受介入治疗;另1例因反复大咯血行右下肺叶切除,目前仍接受小剂量环磷酰胺治疗,病情稳定。结论白塞氏病合并肺血管病变的特点为肺动脉瘤形成,咯血为主要的临床症状,血管造影能明确血管瘤的部位,增强胸部CT能够确定诊断。糖皮质激素加免疫抑制剂是治疗该病的主要手段。

Pulmonary vascular manifestation of Behcet''''s disease:analysis of 7 cases

Objective To investigate the pulmonary vascular features of Behcet's disease in order to improve the recognition and the treatment of this disease at early stage.Methods Seven cases diagnosed as Behcet's disease with pulmonary vascular involvement from 1999 to 2004 were analyzed retrospectively including clinical manifestations,radiological features and pathological characteristics.Results Haemoptypsis of varying degree was the typical symptom of pulmonary vascular involvement of Behcet's disease (7/7).Radiological images demonstrated hilar enlargement and well-marginated round opacities at varied size in the peripheral lung regions(7/7).Pulmonary angiography disclosed pulmonary artery aneurysm at the involved regions(5/5).Pathology of pulmonary vessel from a pneumonectomy sample showed a neutrophilic vasculitis.Six patients administrated by the combination of predisone and cyclophosphamide had good prognosis in which two cases had underwent interventive treatment.One case received pneumonectomy due to major haemoptysis caused by Behcet's disease is now stable with the treatment of cyclophosphamide.Conclusions Pulmonary vessel manifestation of Behcet's disease is formation of pulmonary artery aneurysm and haemoptysis is a prominent complaint.Pulmonary angiography could be valuable to make definite diagnosis.Empirical anti-inflammatory and/or immunosuppressive agents are the mainstay of treatment.