具有自身免疫特征的间歇性肺炎的特征和危险因素
Sana Vahidy1, Jonathan Agyeman2, Boyang Zheng3
1Department of Medicine, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
具有自身免疫特征的间歇性肺炎 (IPAF) 患者的存活率比结缔组织疾病相关的ILD (CTD-ILD) 糟糕,但与无法分类的ILD相似. 肺功能随着治疗而稳定,但IPAF.
科学领域:
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
背景情况:
- 具有自身免疫特征的间歇性肺炎 (IPAF) 呈现出结缔组织疾病相关的间歇性肺病 (CTD-ILD) 的特征,但缺乏特定CTD的标准.
- IPAF与CTD-ILD和不可分类的ILD共享特征,需要进行比较分析.
研究的目的:
- 为了比较IPAF的基线特征,存活率和治疗反应与CTD-ILD和不可分类的ILD.
- 研究IPAF的独特临床轨迹和治疗结果.
主要方法:
- 从一个前性注册表中提取数据,将IPAF (n=128) 与CTD-ILD (n=1240) 和无法分类的ILD (n=665) 进行比较.
- 线性混合模型分析了mycophenolate或azathioprine启动前后的肺功能轨迹 (FVC%,DLCO%).
主要成果:
- 与CTD-ILD相比,IPAF患者年龄更大,男性更多,吸烟史更大. 与无法分类的ILD相比,IPAF患者年轻,女性多,肺功能基线较差.
- IPAF的死亡率高于CTD-ILD,死亡率与未分类的ILD相似.
- 在大多数ILD亚型中,mycophenolate稳定了肺功能,IPAF中的FVC%除外. 阿扎西奥普林稳定了肺功能,除了FVC%的IPAF下降和CTD-ILD的DLCO%的下降.
结论:
- IPAF患者的生存率比CTD-ILD更差,死亡率与未分类ILD相似.
- 用mycophenolate或azathioprine治疗通常稳定了ILD亚型的肺功能.
- IPAF作为一个独立的ILD子集团的分类仍然不确定.
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