Gonon M, Solèr M, Bolliger C T, Imhof E, Elsasser S, Dalquen P, Steinbrich W, Grädel E, Perruchoud A P
Abteilung für Pneumologie, Universitätsspitals Basel.
Dtsch Med Wochenschr. 1993 Apr 2;118(13):457-62. doi: 10.1055/s-2008-1059349.
A 27-year-old man, in good health but a moderate smoker, suddenly had two episodes of haemoptysis. Routine clinical examination was unremarkable. Erythrocyte sedimentation rate was increased to 34 mm/h. The chest radiography showed ill-defined, contrast-poor infiltrations bilaterally, as well as left hilar enlargement. Lung scintigraphy and pulmonary arteriogram suggested pulmonary embolism, possible from a "pelvic vein spur", i.e. an intimal proliferation due to crossing of the common iliac artery over the pelvic vein. He was placed on oral anticoagulants. Three months later he had another severe haemoptysis, providing the indication for an exploratory thoracotomy. This revealed the left pulmonary artery wall to have inflammatory changes with aneurysmal dilatation. The aneurysm was plicated. Histological examination demonstrated chronic vasculitis as seen in Behçet's syndrome, a diagnosis confirmed by the findings of ulcers of the oral mucosa and the presence of HLA B5 allo-antigens. Immunosuppressive treatment was given with prednisone (1 mg/kg), azathioprine (2.5 mg/kg) and ciclosporin (5 mg/kg). Over the next 12 months there has been only one further haemoptysis.
一名27岁男子,身体健康,但吸烟量中等,突然出现两次咯血。常规临床检查无异常。红细胞沉降率增至34mm/h。胸部X线片显示双侧边界不清、对比度差的浸润影,以及左肺门增大。肺部闪烁扫描和肺动脉造影提示肺栓塞,可能源于“盆腔静脉嵴”,即由于髂总动脉横跨盆腔静脉导致的内膜增生。他开始口服抗凝剂。三个月后,他再次出现严重咯血,这为进行 exploratory thoracotomy(此处可能有误,推测是 exploratory thoracotomy,即开胸探查术)提供了指征。手术发现左肺动脉壁有炎症改变并伴有动脉瘤样扩张。对动脉瘤进行了折叠处理。组织学检查显示为白塞病所见的慢性血管炎,口腔黏膜溃疡及 HLA B5 同种抗原的存在证实了这一诊断。给予泼尼松(1mg/kg)、硫唑嘌呤(2.5mg/kg)和环孢素(5mg/kg)进行免疫抑制治疗。在接下来的12个月里,仅又出现过一次咯血。