特发性肺纤维化和进行性肺纤维化。
Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis.
机构信息
Section of Pulmonary and Critical Care, Department at University of Chicago, 5841 South Maryland Avenue, MC 6076, Chicago, IL 60637, USA.
Interstitial Lung Disease Program, Section of Pulmonary/Critical Care, University of Chicago, Chicago, IL, USA.
出版信息
Immunol Allergy Clin North Am. 2023 May;43(2):209-228. doi: 10.1016/j.iac.2023.01.010. Epub 2023 Mar 3.
Idiopathic pulmonary fibrosis (IPF), a common interstitial lung disease (ILD), is a chronic, progressive fibrosing interstitial pneumonia, with an unknown cause. IPF has been linked to several genetic and environmental risk factors. Disease progression is common and associated with worse outcomes. Management often encompasses pharmacotherapy, supportive interventions, addressing comorbidities when present, and treating hypoxia with ambulatory O2. Consideration for antifibrotic therapy and lung transplantation evaluation should occur early. Patients with ILD other than IPF, and who have radiological evidence of pulmonary fibrosis, may have progressive pulmonary fibrosis.
特发性肺纤维化（IPF），一种常见的间质性肺疾病（ILD），是一种原因不明的慢性、进行性纤维性间质性肺炎。IPF 与几个遗传和环境危险因素有关。疾病进展常见，且与预后较差相关。管理通常包括药物治疗、支持性干预、治疗共存疾病和用便携式氧气治疗低氧血症。应早期考虑抗纤维化治疗和肺移植评估。除 IPF 以外的ILD 患者，如果有肺纤维化的放射学证据，可能患有进行性肺纤维化。
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