Analysis of clinical features and prognostic factors in Takayasu arteritis involving pulmonary hypertension: A retrospective study

Abstract

Background: Multiple takayasu arteritis (TA) is a chronic nonspecific large to medium vasculitis disease that mainly accumulates the aorta and its branches. Pulmonary vascular disease is often seen as stenosis and occlusion, and patients may show no moderate to severe pulmonary hypertension (PH). This study aims to summarize the clinical characteristics and analysis of prognostic factors in patients with PH caused by TA.

Methods: Patients diagnosed with aortitis involving the pulmonary artery by pulmonary arteriography or pulmonary artery and total aortic computed tomography arteriography (CTA). All patients underwent detailed clinical assessment, laboratory data collection, and analysis of imaging data. Patients were followed up and factors affecting the prognosis of the pulmonary arteries were analyzed.

Results: Most of the patients' complaints were chest tightness, shortness of breath, decreased activity tolerance, hemoptysis and chest pain. 56.90% of the patients were in at the time of admission. Echocardiographic estimation of pulmonary artery systolic pressure was 90.39 ± 22.87 mm Hg. In terms of laboratory tests, 39.66%% of the patients had elevated C-reactive protein and erythrocyte sedimentation rate, and amino-terminal natriuretic peptide precursor on admission. In terms of imaging, all patients had pulmonary artery involvement, which was combined with aortic involvement in 31.03%. Nuclide lung perfusion/ventilation imaging of the patients revealed multiple perfusion defects/absences in the segmental and subsegmental distribution of the lungs. Univariate Cox regression model analysis suggested that patients' WHO functional class at admission, age ≧ 51 years at the time of consultation, and amino-terminal natriuretic peptide precursor ≧ 3500 pg/mL were factors affecting the prognosis. Further multifactorial Cox regression model analysis suggested amino-terminal natriuretic peptide precursor ≧ 3500 pg/mL was an independent predictor of poor prognosis with a hazard ratio (HR) value of 5.248.

Conclusion: Electrocardiogram and echocardiogram may suggest an increased right heart load; some patients have elevated serum inflammatory indexes. Characteristic imaging manifestations include widening of the main pulmonary artery, multiple pulmonary segmental and subsegmental stenoses.

Figure 1.

Pulmonary CT and aortic CTA. (A,B) Pulmonary CT showed ground glass exudation scattered in the right upper lung and considered the bleeding focus. (C) Subpleural cuneiform shadow. (D) Pulmonary enhanced CT showed the complete occlusion of the right pulmonary trunk and PAH. (E,F) The right bronchial artery was twisted around the right bronchus, forming a collateral circulation. (G) Color Doppler ultrasound of cervical vessels showed the diffuse and centripetal thickening of the wall and stenosis of the lumen from the beginning to the middle and distal segment of the left common carotid artery. (H,I) MRA showed the thickening of the left common carotid artery wall and a narrow lumen, and considered TA (inactive phase). CT = computed tomography, CTA = computed tomography angiography, MRA = magnetic resonance enhanced carotid angiography, PAH = pulmonary arterial hypertension.

Figure 2.

CTA and 3D reconstruction imaging results. (A) Lung CT showed cavitary lesions in the apical segment of the right upper lobe. (B,D,E) Nodular consolidation in the upper lobe of the right lung. (C) Subpleural cuneiform shadow. (F) Following a percutaneous lung puncture, the lung tissue was sent for examination and the pathology results showed coagulative necrosis, the proliferation of surrounding lung tissue, and hemosiderin deposition (a small amount of old bleeding), and the histology indicated pulmonary infarction. The pathogenic test results were negative (Periodic Acid-Schiff stain, ×40). Following CTA of the thoracic aorta, ventral aorta, iliac artery, femoral artery, popliteal artery, tibial and fibular artery a diagnosis of (active phase) TA was considered. The brachiocephalic trunk (G), bilateral subclavian arteries (G), abdominal aorta and celiac trunk (H), superior mesenteric artery (I), and involved bilateral renal arteries (J). CTA = computed tomography angiography, CT = computed tomography, TA = Takayasu arteritis.

Figure 3.

CT and CTPA imaging results of the lung. (A–D) The pulmonary infarction focus. (E) Wall thickening and lumen stenosis of the main right pulmonary artery. (F–H) Right middle lobe and lower lobe pulmonary artery branch occlusion. CT = computed tomography, CTPA = CT pulmonary angiogram.

Figure 4.

PET-CT imaging results of a patient with TA. PET-CT = positron emission tomography-computed tomography, TA = Takayasu arteritis.