Two cases of airway-centered fibroelastosis treated with an antifibrotic agent and corticosteroids

Abstract

Airway-centered fibroelastosis is characterized by peribronchovascular fibroelastosis, predominantly in the upper lobes, with little-to-no pleural involvement. In this study, we describe two cases of airway-centered fibroelastosis diagnosed based on radiological and pathological findings. The first case comprised a 44-year-old man whose forced vital capacity improved over three months following treatment with nintedanib. The second case involved a 50-year-old woman who was treated with oral corticosteroids but yielded an unfavorable outcome. An effective treatment for airway-centered fibroelastosis has not yet been identified; therefore, this study may help contribute to a more thorough discussion regarding treatment strategies for this disease.

Keywords

• Airway-centered fibroelastosis
• Pleuroparenchymal fibroelastosis
• Nintedanib
• Corticosteroids
• Progressive fibrosing interstitial lung disease