文献简介

出版社:Clinical and epidemiological research

作  者:Ragnar Gunnarsson,Trond Mogens Aaløkken,Øyvind Molberg

编  号:

关键字:mixed connective tissue disease | a study | interstitial lung disease

年  份:2012   点击量:656

文献摘要

Background Mixed connective tissue disease (MCTD) is an immune-mediated, systemic disorder of unknown cause.研究背景

混合性结缔组织病(MCTD)是一种免疫介导的、原因不明的全身性疾病。

Objective To assess the prevalence, pattern and severity of interstitial lung disease (ILD) in a crosssectional study of the nationwide, Norwegian MCTD cohort.

研究目的

挪威MCTD队列在全国范围内探讨间质性肺疾病(ILD)的的流行、模型和严重性。

Methods 126 patients with MCTD were systematically examined for ILD by high-resolution CT (HRCT), pulmonary function tests (PFT), 6 min walk test (6MWT) and by the New York Heart Association (NYHA) functional classification of dyspnoea. The extent and type of HRCT lung abnormalities were scored according to the CT criteria of ILD recommended by the Fleischner Society.

研究方法

我们通过高分辨率CT ( HRCT )、肺功能检查( PFT )、6分钟步行试验( 6MWT )并采用纽约心脏病学会(NYHA )的呼吸困难功能分级,对126个MCTD患者进行了LED的系统性检查。根据Fleischner 学会推荐的ILDCT标准来评估HRCT肺部异常情况的程度和类型。

Results All 126 patients were Caucasian, 75% women. At the time of the cross-sectional ILD study, the patients had a mean disease duration of 9.0 years. 52% of the patients had abnormal HRCT findings, most commonly reticular patterns consistent with lung fibrosis (35%). Lung fibrosis was quantified as minor in 7%, moderate in 9% and severe in 19% of the patients. Fibrosis was uniformly concentrated in the lower parts of the lungs and was not associated with smoking. Patients with severe lung fi brosis had lower PFT values, shorter 6MWT and a higher mean NYHA functional class. After a mean 4.2 years’ follow-up, overall mortality was 7.9%. Mortality in patients with normal HRCT was 3.3%, as compared with 20.8% in patients with severe lung fibrosis (p<0.01).

研究结果

所有的126名患者都为白人,其中75%为女性。在对ILD的横断面研究时,患者的平均病程为9.0年。52%的患者有异常HRCT表现:肺纤维化(35%)最常见的网状图案。我们队肺纤维化量化:轻微患者为7%,中度患者为9%,重度患者为19%。纤维化均匀地集中在肺的下部,并与吸烟无关。重度肺纤维化患者的PFT值较低,6MWT较短且NYHA功能分级平均值较高。通过平均4.2年的随访后,总死亡率为7.9%。与重症患者的肺纤维化(P <0.01)患者的20.8%(死亡率)相比,正常的HRCT患者死亡率为3.3%。

Conclusions Severe lung fibrosis is common in MCTD, has an impact on pulmonary function and overall physical capacity and is associated with increased mortality.

研究结论

严重的肺纤维化常见于MCTD,其对肺功能和体质的造成影响,并且与死亡率的增加有关。