混合结合性组织疾病患者的表型,其中有相关的间歇性肺病
Gonçalo Boleto1, Silje Reiseter2, Anna-Maria Hoffmann-Vold3
1Department of Rheumatology, Université Paris Cité, Cochin Hospital, Paris, France; Instituto Português de Reumatologia, Lisboa, Portugal.
这项研究确定了与混合结合组织疾病 (MCTD) 中的间歇性肺病 (ILD) 相关的因素. 数字预测肺功能下降,MCTD-ILD患者面临更高的死亡率.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 免疫学 免疫学 免疫学
背景情况:
- 混合结合组织疾病 (MCTD) 可以影响肺部,导致间歇性肺病 (ILD).
- 在MCTD中预测ILD进展和严重程度对于患者管理至关重要.
研究的目的:
- 为了比较有和没有ILD的MCTD患者.
- 确定与ILD发展,进展和严重程度相关的因素.
主要方法:
- 一项国际多中心追溯研究,对300名MCTD患者进行了研究.
- 基于高分辨率计算机断层扫描 (HRCT) 和强迫生命能力 (FVC) 测量的ILD诊断.
- 在基线和随访期间评估与ILD相关的因素.
主要成果:
- 年龄较大,皮肤变厚,胃肠道症状,肺功能减弱 (FVC,DLCO),特异性自身抗体 (抗毒素酶,SSA/Ro),冷球蛋白血症和CRP升高与ILD存在有关.
- 菌酸莫菲蒂尔经常用于MCTD-ILD患者.
- 数字 (DU) 是FVC衰退的危险因素,而MCTD-ILD患者的死亡率更高.
结论:
- 几个因素可以预测MCTD患者的ILD.
- 数字性与MCTD中渐进的肺病有关.
- 患有ILD的MCTD患者的死亡率增加.
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