摘要
目的探讨混合性结缔组织病(MCTD)相关性肺部病变的临床特点、诊断、治疗方法及预后。方法对哈尔滨医科大学附属第二医院呼吸科1996年1月至2008年6月入院治疗的112例诊断明确的MCTD患者进行回顾性分析,对其临床表现、放射学、心脏彩超及肺功能进行观察。对其中44例患者进行随访。结果112例MCTD患者中肺部有病变者48例(42.8%),其中女43例。主要症状是气短(11.6%)、胸痛(3.6%)和咳嗽(3.6%)。48例肺部存在病变患者中,25例患者肺功能下降和超声影像学异常时可无任何呼吸系统症状(52.1%)。98例MTCD患者中,影像学异常者41例(41.8%),常见的是胸腔积液15例(36.6%)和胸膜肥厚6例(14.6%)。45例行肺功能检查30例异常,其中一氧化碳弥散量(DLCO)异常22例(73.3%)。78例行超声心动图检查,发生肺动脉高压(PAH)者8例(10.3%)。随访7例发生PAH患者,3例死亡。结论MCTD肺部病变不少见,严重影响预后,DLCO是最敏感的评价MCTD患者肺功能下降的参数,PAH是混合性结缔组织病最常见的死亡原因。
Objective To study the clinical manifestations, diagnosis, treatments and prognosis of the pulmonary change in mixed connective tissue disease(MCTD). Methods From January 1996 to June 2008,112 patients with definite MCTD in Second Affiliated Hospital of Harbin Medical University were retrospectively studied, and the clinical feature about pulmanary manifestations, eehocardiography feature, radiology features and treatments were reviewed and analyzed. 44 patients were followed up. Results 48(5 male)had pulmonary manifestation. The median age of onset of symptoms was 39 years and the median duration of disease was three years. The most common manifestation was pleural effusion and(or) paehynsis pleurae(21 eases,18. 8% ). 16 patients( 14. 3% ) had interstitial lung disease,and 8 cases(7. 1% )had pulmonary hypertension. The cardinal symptom was short breath. Thirteen patients ( 11.6% ) had dyspnea,4 cases ( 3.6% ) had chest pain,and 4 cases( 3.6% )had cough. Reductions of pulmonary function or abnmTnalities of uhrasonographic image were noted in 25 ( 52. 1% ) of 48 patients with pulmonary involvement of MCTD who had not any complaint of respiratory symptoms. Six patients had reductions in diffusing capacity of the lung for carbon monoxide(DLCO) , 17 patients had pulmonary abnormality on chest X ray or CT, and two patients had pulmonary hypertension on doppler eehocardiography. Conclusion Pulmonary involment of MCTD may contribute to prognosis seriously. DLCO was the most sensitive parameter. Pulmonary hypertension was the most common cause of death.
出处
《中国实用内科杂志》
CAS
CSCD
北大核心
2009年第9期827-829,共3页
Chinese Journal of Practical Internal Medicine
基金
黑龙江省教育厅科学技术研究项目(11521096)
关键词
混合性结缔组织病
肺疾病
肺功能
mixed connective tissue disease
pulmonary manifestations
pulmonary function