摘要
目的：分析白塞病（ BD）合并肺高压（ PH）的临床特点。方法回顾性分析2000年1月至2015年8月北京协和医院住院的BD合并PH患者的病因、临床特点、治疗情况及预后等临床资料。结果 BD合并PH 25例，约占同期BD住院患者（912例）的3％。25例中男性15例（60％），女性10例（40％），年龄19～66岁，平均（33±12）岁。发现PH与确诊BD时间间隔为0～40年，中位时间1年。 PH的病因为：心脏瓣膜病变10例（40％），肺动脉狭窄或闭塞6例（24％），肺动脉瘤合并肺血栓形成1例（4％），单纯肺血栓1例（4％），心肌病变1例（4％），病因不明6例（24％）。超声心动图估测肺动脉收缩压为40～117 mmHg（1 mmHg＝0．133 kPa），平均（60±22） mmHg。诊断BD合并PH时，ESR升高者占48％（11／23），超敏C反应蛋白（hs－CRP）升高者占82％（14／17）。经激素（96％，24／25）、免疫抑制剂（92％，23／25）、抗凝或溶栓（36％，9／25）以及抗肺动脉高压靶向药物（32％，8／25）治疗后，ESR下降10例（10／11），hs－CRP下降10例（10／14），复测肺动脉收缩压降低者8例（8／16）。9例随访2～96个月，死亡4例，恶化1例，稳定2例，好转1例。结论 PH是BD少见的并发症，其病因主要为瓣膜病变和肺动脉受累，治疗效果欠佳，预后较差。
Objective To understand the clinical features of Behcet′s Disease（ BD） with pulmonary hypertension（ PH） .Methods The etiology, clinical features, treatment and prognosis of 25 patients with Bahcet′s disease （ BD） complicated with pulmonary hypertension （ PH） admitted in Peking Union Medical College Hospital from January 2000 to August 2015 were retrospectively reviewed.Results Total 912 BD patients were hospitalized during the same period, among whom 25 cases were complicated with PH accounting for 3%.There were 15 males （60%） and 10 females （40%）, with the mean age of （33 ±12） years （range 19 to 66 years）.The median interval from the diagnosis of BD to the onset of PH was 1 year （range 0 to 40 years）.The most common cause of PH was heart valve disease （n=10, 40%）, followed by pulmonary arterial stenosis or occlusion （n=6, 24%）, pulmonary arterial aneurysm with thrombus （n=1, 4%）, pulmonary thrombosis （n=1, 4%）, cardiomyopathy （n=1, 4%）;there were no causes identified in 6 cases （24%）.Pulmonary arterial pressures estimated by echocardiography were 40 to 117 mmHg （1 mmHg=0.133 kPa） with a mean pressure of （60 ±22） mmHg.When the PH developed, elevated erythrocyte sedimentation rate （ ESR ） and increased hypersensitive C-reactive protein （ hs-CRP ） were founded in 48% （ 11/23 ） and 82% （ 14/17 ） of patients, respectively.After treated with glucocorticoid （96%,24/25）, immunosuppressive agents（92%, 23/25）, anticoagulation or thrombolysis（36%,9/25） and specific targeted vasodilator（32%,8/25）, the levels of ESR and hs-CRP declined in 91% （10/11） and 71%（10/14） of patients, respectively; and pulmonary arterial systolic pressure declined in 50% of＆amp;nbsp;cases （8/16）.Among 9 patients followed for 2 to 96 months, 4 died, 1 aggravated, 2 kept stable and 1 improved.Conclusion PH is an uncommon complication in disease.Heart valve disease, pulmonary artery involvement are the major causes of PH.The therapeutic effect and prognosis are poor.
出处
《中华全科医师杂志》
2016年第10期770-774,共5页
Chinese Journal of General Practitioners
关键词
贝赫切特病
肺动脉高压
Behcet's disease
Pulmonary hypertension
