Evaluation of clinical practice guidelines on treatment of cystic fibrosis: A systematic review.

Feb 17, 2025

Experts: Yuting Huang,Jingxuan Zhang,Mianquan Zhang,Xuetao Kong,Zhufeng Wang,Yuxiang Zhang,Zhili Zou,Zhuyinjun Zong,Jiaying Guo,Quanzhen Liu,Jing Ling,Wangji Zhou,Xueqi Liu,Jie Liu,Xinlun Tian,Mei Jiang

Despite the existence of numerous clinical practice guidelines (CPGs) for cystic fibrosis (CF), there is limited understanding of their credibility and consistency. This systematic review aims to comprehensively evaluate the quality of CPGs for CF and its pulmonary complications, focusing on treatment recommendations for pulmonary care.
We conducted a comprehensive search across four databases and relevant websites to identify eligible guidelines providing treatment recommendations. The quality of these guidelines was assessed using the Appraisal of Guidelines for Research and Evaluation (AGREE) II tool. Pulmonary treatment recommendations were analyzed and synthesized narratively.
A total of 35 guidelines were identified. Most guidelines were of moderate quality according to the AGREE II instrument, with overall scores ranging from 21·05 to 76·13. Only six guidelines were recommended for use. These guidelines provide 359 pulmonary treatment recommendations for seven primary therapies and others. There was inconsistency in the use of airway clearance therapy, anti-inflammatories, antibiotics, inhaled drugs, and cystic fibrosis transmembrane conductance regulator modulator therapy. Four guidelines conditionally advocated for oral corticosteroids, while six opposed routine inhaled corticosteroids. One guideline discouraged lumacaftor-ivacaftor in the general CF population, two recommended only for children under 12 years old, and another strongly advocated for children between 2 and 5 years of age. However, one guideline noted a lack of evidence to recommend it for children under 6.
The quality of CPGs for CF and its pulmonary complications has improved over time, reaching a moderate level generally, but there is still room for further improvement. Future efforts should focus on standardizing methodological frameworks and generating robust clinical evidence to enhance the overall quality and applicability of CF guidelines.

Copyright © 2025 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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ABOUT THE EXPERTS

Yuting Huang,Jingxuan Zhang,Mianquan Zhang,Xuetao Kong,Zhufeng Wang,Yuxiang Zhang,Zhili Zou,Zhuyinjun Zong,Jiaying Guo,Quanzhen Liu,Jing Ling,Wangji Zhou,Xueqi Liu,Jie Liu,Xinlun Tian,Mei Jiang

Yuting Huang

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China.

Jingxuan Zhang

Nanshan School, Guangzhou Medical University, Guangzhou, Guangdong, China.

Mianquan Zhang

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China.

Xuetao Kong

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China.

Zhufeng Wang

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China.

Yuxiang Zhang

Nanshan School, Guangzhou Medical University, Guangzhou, Guangdong, China.

Zhili Zou

The First School of Clinical Medicine, Guangzhou Medical University, Guangzhou, Guangdong, China.

Zhuyinjun Zong

Nanshan School, Guangzhou Medical University, Guangzhou, Guangdong, China.

Jiaying Guo

The First School of Clinical Medicine, Guangzhou Medical University, Guangzhou, Guangdong, China.

Quanzhen Liu

Nanshan School, Guangzhou Medical University, Guangzhou, Guangdong, China.

Jing Ling

Guangdong Cardiovascular Institute, Guangdong Provincial People’s Hospital, Guangzhou, China.

Wangji Zhou

Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Xueqi Liu

Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Jie Liu

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China. Electronic address: ljgird001@163.com.

Xinlun Tian

Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. Electronic address: tianxl@pumch.cn.

Mei Jiang

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong, China. Electronic address: jiangmei927@163.com.