B. Shine Rheumatology Unit, Rambam Health Care Campus, Haifa, Israel.
Semin Arthritis Rheum. 2011 Dec;41(3):461-70. doi: 10.1016/j.semarthrit.2011.06.001. Epub 2011 Jul 30.
To review pulmonary arteritis (PA) complicated by pulmonary arterial hypertension (PAH) in Takayasu's arteritis (TA).
Two cases of PA and PAH in TA patients and similar cases published in the Medline database from 1975 to 2009 were reviewed.
Forty-six cases (females 89.1%, Asians 65%, mean age 34.6 years) were analyzed, 42.2% of which had PAH. Isolated PA was reported in 31.8%. Respiratory symptoms were presented as dyspnea (75.5%), chest pain (48.9%), hemoptysis (42.2%), and cough (17.7%). Hypertension, vascular bruits, and diminished/absent pulses were reported in 48.9% of patients. A diagnosis of PA was based on abnormal uptake on pulmonary perfusion scan and a finding of stenosis, narrowing, occlusion, and irregularity on computed tomography or magnetic resonance imaging, and/or pulmonary angiography. Patients were treated with glucocorticoids (77.5%), disease-modified antirheumatic drugs (35%), and warfarin (20%); only a few were treated with biological agents. Vascular procedures were performed in 52.5% of cases, on pulmonary arteries in 37.5% with good results. The outcome was death in 20.5% of PA patient and 33.3% in PAH patients.
TA may be complicated by life-threatening PA and PAH. Clinical signs are not specific and may be masked by involvement of the aorta and its branches. Treatment with glucocorticoids and disease-modified antirheumatic drugs has only partial effect, which may be intensified by biological agents. Invasive procedures on pulmonary arteries may be a complementary option. PA and PAH in TA patients should be recognized early and treated promptly for prevention of irreversible vascular damage.
综述大动脉炎（TA）并发肺动脉炎（PA）和肺动脉高压（PAH）。
回顾分析 TA 患者并发 PA 和 PAH 的 2 例病例及 2009 年以前 Medline 数据库中类似病例。
共分析 46 例患者（女性占 89.1%，亚洲人占 65%，平均年龄 34.6 岁），42.2%合并 PAH，孤立性 PA 占 31.8%。主要临床表现为呼吸困难（75.5%）、胸痛（48.9%）、咯血（42.2%）和咳嗽（17.7%）。48.9%的患者有高血压、血管杂音和脉搏减弱或消失。PA 的诊断依据是肺灌注扫描摄取异常、CT 或 MRI 显示狭窄、变窄、闭塞和不规则，以及/或肺动脉造影。患者接受糖皮质激素（77.5%）、改善病情抗风湿药（35%）和华法林（20%）治疗，少数患者接受生物制剂治疗。52.5%的患者接受了血管介入治疗，37.5%的患者对肺动脉进行了介入治疗，效果良好。PA 患者中有 20.5%死亡，PAH 患者中有 33.3%死亡。
TA 可并发危及生命的 PA 和 PAH，临床症状无特异性，可能被主动脉及其分支受累所掩盖。糖皮质激素和改善病情抗风湿药治疗效果有限，生物制剂可能会增强疗效。肺血管介入治疗可能是一种补充治疗方法。TA 患者的 PA 和 PAH 应早期诊断并及时治疗，以防止不可逆的血管损伤。
