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Airway-centered fibroelastosis identified as distinct entity

Researchers in France identified progressive airway-centered disease as a distinct entity occurring in non-smoking women aged between 40 and 60 years, according to recent research.

The disease is characterized by extensive upper lobe airway-centered fibroelastosis and bronchial abnormalities, which includes bronchial wall deformation and thickening as well as bronchiectasis. Other characteristics include progressive parenchymal retraction and subpleural upper-lobe consolidations.

“The identification of airway-centered fibroelastosis as a distinct entity, together with [pleuropulmonary fibroelastosis (PPFE)], identifies elastosis as a neglected pathway of lung remodeling and suggests that lung elastosis may cover a spectrum of diseases,” Pauline Pradere, MD, of the University Paris Diderot, and colleagues wrote. “Collaborative studies are needed to better delineate the prevalence of the disease, identify pathophysiological pathways and develop therapeutic weapons.”

The researchers described five patients aged between 38 and 56 years who had chronic dyspnea and acute attacks of dyspnea and wheezing, according to the abstract. Although having similar characteristics to PPFE, these patients had clinical, radiological, pathological and physiological differences from patients with PPFE.

“All our patients had acute exacerbations of cough, dyspnea, and wheezing, which are not observed in PPFE,” Pradere and colleagues wrote. “In PPFE, patients usually have dyspnea on exertion and/or cough without acute deterioration of symptoms.”

Pradere and colleagues also noted differing lung function tests, with three patients having an obstructive pattern that would normally present as a restrictive pattern in patients with PPFE. Similarly, high resolution CT scans of patients with airway-centered fibroelastosis showed upper lobe volume loss with distortion, but without pleuroparenchymal thickening normally present in patients with PPFE.

“By contrast, bronchiolar and bronchial changes were prominent, with bronchial wall thickening, bronchial wall deformation, bronchiectasis, and marked peribronchial consolidations, which are unusual in PPFE,” the researchers wrote.

Pradere and colleagues noted respiratory failure occurred in all five patients despite use of inhaled and oral corticosteroids, with 2 patients ultimately undergoing lung transplantation. – by Jeff Craven

Disclosure: The researchers report no relevant financial disclosures.