论文摘要：
Introduction pulmonary takayasu arteritis (PTA) patients are usually diagnosed until very late stage due to the unspecific symptoms or misdiagnosis as chronic thromboembolic pulmonary hypertension (CTEPH). It is important to manifest the clinical presentation and radiologic features of PTA.
Method 61 PTA patients were collected at Beijing ChaoYang Hospital from 2011 to 2016. Patients were classified as group PA-PH (PTA with pulmonary hypertension) and group PA (PTA without pulmonary hypertension). The clinical data including characteristics,
ESR, C-reactive protein, N-terminal pro-brain natriuretic peptide, Computed Tomography Pulmonary Angiography, Pulmonary angiography and right cardiac catheterization were analyzed respectively.
Results 25 patients were in group PA, 36 patients were in group PA-PH. Duration time from onset of symptoms to diagnosis of group PA-PH were longer than that of group PA [18(6,72) months vs. 6(2.5,24) months, P=0.024]. In group PA, hemoptysis, cough and chest pain were top 3 symptoms with frequency of 52%, 52% and 44% respectively, while in group PA-PH, chest tightness, dyspnea and hemoptysis were top 3 symptoms with frequency of 81%, 56% and 47% respectively. The occurrence of chest tightness, dyspnea in group PA-PH were higher that of group PA [29,(81%)vs. 10,(40%) ,p=0.001 and 32,(56%)vs. 9,(36%),p=0.000]. Imaging results showed that PTA patients have 5 kinds of arteries lesions: occlusion, thickening of vascular wall, stenosis, in situ thrombosis and ectasis /aneurysm. Prevalence of thickening of vascular walls of group PA was higher than that of group PA-PH [21/25(84%) vs. 13/36(36%), p=0.000], and it was common to find that different kinds of arteries lesions occurred simultaneously in one patient: 33% patients had 2 kinds of arteries lesions combined, 23% patients had 3 kinds of arteries lesions combined, 23% patients had 4 kinds of arteries lesions combined. Rat-tail stenosis was a specific artery lesion for PTA and occurs on 15 patients’ right pulmonary artery trunk.
Conclusion PTA has specific clinical and imaging characteristics, and different manifestations between patients with or without pulmonary hypertension. This may help to identify PTA patients earlier in clinical work.
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