摘要
目的总结多发性大动脉炎累及肺动脉所致肺动脉高压患者的临床特征。方法收集阜外医院1988年6月至2014年6月多发性大动脉炎累及肺动脉患者94例，根据主动脉有无受累分为主动脉及肺动脉均受累（APTA组，48例）和单独肺动脉受累（PTA组，46例）；分析其临床及血管影像学特征。结果患者男女比例1：3．3，发病时年龄8～60岁，中位年龄33．5岁。主要临床表现为劳力性气短；体征包括心脏杂音及收缩中期肺野血管杂音。超声心动图检查提示所有患者均有肺动脉高压；病变多累及肺叶、段动脉，其次为亚段血管；双侧多于单侧，右侧多于左侧。APTA组与PTA组的临床症状、体征无显著差异。而入院时APTA组的红细胞沉降率和N末端脑钠肽原均显著高于PTA组[14．0（5．0，28．0）比8．5（3．0，19．3）mm／1h和（1936±1769）比（1627±1153）ng／L，均P〈0．05]。结论多发性大动脉炎累及肺动脉患者以气短为主诉，就诊时多有肺动脉高压，并伴中重度心功能不全。
Objective To summarize the clinical features of Takayasu arteritis （TA） patients with pulmonary hypertension due to pulmonary artery involvement. Methods Ninety-four TA patients with pulmonary artery involvement treated in Fuwai Hospital from Jun 1988 to Jun 2014 were retrospectively summarized. Patients were divided into two groups according to whether aorta and its main branches affected （ APTA, n = 48 ） or not （ PTA, n = 46 ）. Clinical features and angiographic data were further analyzed. Results Of all the patients, male to female rate was 1 ： 3.3, age ranged from 8 to 60 years with median age of 33.5 years when symptom onset. Main symptoms included dyspnea on exertion. Heart murmur and mid- systolic murmur in pulmonic area were commonly found by physical examination. All patients had pulmonary hypertension by echocardiographic examination. Angiographic data showed that multi-lobular and multi- segmental pulmonary branches were predominantly affected, followed by sub-segmental and peripheral branches. Both sides of pulmonary artery involvement were more common than one side involvement, while the right pulmonary arteries were more often affected than the left. Most of the clinical symptoms and signs between APTA group and PTA group were comparable. However, erythrocyte sedimentation rate and N- terminal pro-natriuretic peptide were significant higher in APTA group than that in PTA group when admission [ 14. 0（5.0,28.0） vs 8. 5（3.0,19.3） mm/1 h and （ 1 936 ±1 769） vs （ 1 627 ± 1 153） ng/L, both P 〈 0. 05 ]. Conclusions Dyspnea on exertion may be the main manifestation of TA with pulmonary artery involvement. All patients present with puhnonary hypertension and moderate to severe heart failure when symptom onset.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2016年第16期1252-1255,共4页
National Medical Journal of China
基金
国家科技支撑计划（2011BAI00B15）
关键词
动脉炎
高血压
肺性
疾病特征
Arteritis
Hypertension, pulmonary
Disease attributes
