Bronchiectasis: literature review for preparation of 2024 clinical guidelines

Abstract

Bronchiectasis, ICD-10 – J47, (BE) is a chronic respiratory disease characterized clinically by cough, sputum production and bronchial infection, and radiographically by abnormal and persistent dilation of the bronchi. Common causes include cystic fibrosis, primary ciliary dyskinesia, immune disorders, systemic inflammatory diseases and infections, and other factors. However, some cases are idiopathic, when the cause cannot be identified. In practice, patients with bronchiectasis are divided into two groups: associated and not associated with cystic fibrosis. The prevalence of the disease varies significantly worldwide; it is not reliably known in the Russian Federation.The aim of the review is to analyze the literature data on modern approaches to the diagnosis of BE and to familiarize readers with diagnostic methods and basic approaches to the treatment.Methods. Data from 77 articles and the expert opinion of specialists providing care to patients with BE were used.Results. The main causes, frequency of occurrence, clinical phenotypes and treatment approaches for BE are described. There are many clinical, laboratory, instrumental and radiological features that provide insight into the etiology of BE. The European consensus is that the goal of treating BE is to restore or maintain normal lung function. There are no randomized trials on the treatment of BE, so all treatment guidelines are based on very low-level evidence or extrapolated from cystic fibrosis guidelines. Recommendations for mucolytic, antibacterial and anti-inflammatory therapy for BE are described, taking into account international and national experience.Conclusion. The development of a new version of clinical guidelines with modern relevant information will improve the diagnosis and treatment of BE in the Russian Federation.