Brugiere O, Mal H, Sleiman C, Groussard O, Mellot F, Fournier M
Service de Pneumologie et Réanimation Respiratoire, Hôpital Beaujon, Clichy, France.
Eur Respir J. 1998 Mar;11(3):767-70.
Pulmonary arteries involvement is well described in Takayasu's arteritis (TA), a condition which is mainly associated with involvement of the systemic arteries. We report a case of TA with documented isolated pulmonary arteries involvement. Symptoms were quite similar to those encountered in chronic thromboembolic disease. A pulmonary angiogram showed bilateral stenosis and occlusion of pulmonary arteries. Diagnosis of TA was suspected, and as such a complete aortogram was made but proved to be normal. Massive haemoptysis suddenly occurred, which resulted in death. Autopsy disclosed characteristic pathological lesions of TA in pulmonary arteries and confirmed the lack of involvement of the aorta and its branches. The frequency of such a clinical form could be underestimated, given the difficulties of diagnosis and features similar to those of chronic thromboembolic disease.
肺动脉受累在大动脉炎(TA)中已有充分描述,TA主要与系统性动脉受累相关。我们报告一例有记录的孤立性肺动脉受累的TA病例。症状与慢性血栓栓塞性疾病所见颇为相似。肺血管造影显示双侧肺动脉狭窄和闭塞。怀疑为TA,因此进行了完整的主动脉造影,但结果正常。突然发生大量咯血,导致患者死亡。尸检发现肺动脉有TA的特征性病理病变,并证实主动脉及其分支未受累。鉴于诊断困难以及与慢性血栓栓塞性疾病相似的特征,这种临床类型的发生率可能被低估。