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特发性肺纤维化

最后审阅: 23 Jun 2024
最后更新: 12 Oct 2022

小结

定义

病史和体格检查

关键诊断因素

  • 存在的危险因素
  • 呼吸困难
  • 咳嗽
  • 爆裂音
完整详情

其他诊断因素

  • 体重减轻、疲劳和乏力
  • 杵状指
完整详情

危险因素

  • 高龄
  • 男性
  • 家族史
  • 吸烟
  • 有机和无机粉尘暴露
  • 胃食管反流
  • 感染
  • 糖尿病
完整详情

诊断性检查

首要检查

  • 胸部 X 线检查 (CXR)
  • 高分辨率CT(HRCT)
  • 抗核抗体(免疫荧光检测)
  • 类风湿因子
  • 抗环瓜氨酸肽
  • 肌炎全套检查
完整详情

需考虑的检查

  • 肺功能检查 (PFT)
  • 外科肺活检
  • 支气管肺泡灌洗 (bronchoalveolar lavage, BAL)
  • 经支气管活检
  • C 反应蛋白 (CRP)
  • 红细胞沉降率 (ESR)
完整详情

治疗流程

急症处理

急性加重

持续性治疗

目前未出现急性加重的患者

撰稿人

作者

Mary Elizabeth Kreider, MD, MS

Associate Professor of Clinical Medicine

Pulmonary, Allergy, and Critical Care Division

Perelman School of Medicine

University of Pennsylvania

Philadelphia

PA

利益声明

MEK is serving as site principal investigator for Bellerophon and United Therapeutics for interstitial lung disease (ILD) studies. Her institution receives payments for those trials.

Jake G. Natalini, MD

Fellow

Pulmonary, Allergy, and Critical Care Division

Perelman School of Medicine

University of Pennsylvania

Philadelphia

PA

利益声明

JGN declares that he has no competing interests.

鸣谢

Dr Mary Elizabeth Kreider and Dr Jake G. Natalini would like to gratefully acknowledge Dr Judd David Flesch, Dr Gregory Tino, and Dr Jeffrey C. Munson, previous contributors to this topic.

利益声明

GT has served as a consultant for InterMune. GT has served as a principal investigator for clinical trials in idiopathic pulmonary fibrosis. JDF amd JCM declare that they have no competing interests.

同行评议者

Stephen Nathan, MD

Medical Director

Lung Transplant & Advanced Lung Disease Program

Inova Fairfax Hospital

Falls Church

VA

利益声明

SN is an author of a reference cited in this monograph.

Athol Wells, MD, FRCP

Professor of Respiratory Medicine

Interstitial Lung Disease Unit

Royal Brompton Hospital

London

UK

利益声明

AW declares that he has no competing interests.

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