Pathophysiology of small airways in idiopathic pulmonary fibrosis (IPF): the silent zone.

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by distorted alveolar structure and reduced lung compliance, and impaired ventilation-perfusion. Small airway disease (SAD) is  often termed a 'quietzone' due to its asymptomatic nature. Around 30-40% of IPF patients exhibit SAD, which is associated with worse prognosis, higher fibrosis and emphysema scores, and elevated mortality risk. We used PubMed and Google Scholar for literature search.

Areas covered: This review explores the pathophysiology of small airways in IPF, focusing on 1. Risk factors, including age, gender, smoking and occupational dust exposure, and ozone. 2. Diagnostic challenges: SAD is difficult to detect through traditional spirometry or high-resolution computed tomography  imaging due to resolution limitations.  3. Early physiological changes of small airways include airway wall thickening, lumen distortion, and reduced terminal bronchioles, preceding microscopic fibrosis, occurs in the early process of IPF. 4. Pathological mechanisms: The review examines the underlying mechanisms driving small airway disease in IPF.

Expert opinion: A comprehensive approach is essential to improve the understanding and management of SAD in IPF. Priorities include identifying therapeutic targets, advanced imaging and functional assessments. Forced oscillation technique should be introduced for early detection for small airway abnormalities in IPF.