Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
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DOI：
10.1164/rccm.202202-0399st
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发表时间：
2022-05-01
影响因子：
24.7
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这份美国胸科学会、欧洲呼吸学会、日本呼吸学会和拉丁美洲协会的指南更新了先前的特发性肺纤维化(IPF)指南，并阐述了IPF以外的间质性肺疾病(ILDS)患者的肺纤维化进展情况。一个委员会由ILD的多学科专家、方法学家和患者代表组成。1)IPF的更新：对IPF的放射学和组织病理学标准进行了协商一致的更新。关于经支气管镜肺冷凝术、基因组分类器测试、抗酸药物和抗反流手术的问题通过系统综述得到信息，并使用建议分级、评估、发展和评估(GRADE)方法以循证建议回答。2)进展性肺纤维化(PPF)：定义进展性肺纤维化，协商一致确定进行性肺纤维化的放射学和生理学指标。关于吡非尼酮和九替达尼的问题是通过系统审查了解的，并使用分级方法以循证建议回答。1)IPF的更新：一项有条件的建议是，在具有适当专业知识的中心，将经支气管镜肺冷冻活检作为外科肺活检的可接受替代方案。没有提出支持或反对基因组分类器测试的建议。针对IPF的抗酸治疗和抗反流手术提出了有条件的建议。2)PPF：PPF被定义为在过去一年内至少出现三项指标中的两项(症状恶化、放射学进展和生理进展)，除IPF外，对于ILD患者没有其他解释。对九替达尼提出了有条件的建议，并建议对吡非尼酮进行更多的研究。本指南中的有条件建议旨在为临床医生做出合理、知情的决定提供基础。
This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana de Tórax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. A committee was composed of multidisciplinary experts in ILD, methodologists, and patient representatives. 1) Update of IPF: Radiological and histopathological criteria for IPF were updated by consensus. Questions about transbronchial lung cryobiopsy, genomic classifier testing, antacid medication, and antireflux surgery were informed by systematic reviews and answered with evidence-based recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. 2) Progressive pulmonary fibrosis (PPF): PPF was defined, and then radiological and physiological criteria for PPF were determined by consensus. Questions about pirfenidone and nintedanib were informed by systematic reviews and answered with evidence-based recommendations using the GRADE approach. 1) Update of IPF: A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF. A conditional recommendation was made for nintedanib, and additional research into pirfenidone was recommended. The conditional recommendations in this guideline are intended to provide the basis for rational, informed decisions by clinicians.