Bronchiectasis Appears to Be Common in Airway-Centered Fibroelastosis, a Possible New Lung Disorder in Women

Bronchiectasis Appears to Be Common in Airway-Centered Fibroelastosis, a Possible New Lung Disorder in Women
Airway-centered fibroelastosis is a potential new disorder identified in women, according to a study recently published in the journal Chest and titled “Airway-centered fibroelastosis: a distinct entity.” Interestingly, the condition seems to involve bronchiectasis, an abnormal widening of the bronchi or their branches. A team of researchers at the University Paris Diderot, Sorbonne Paris Cité in France, identified five non-smoking women between the ages of 38 and 56 who tested positive for airway-centered fibroelastosis, referring to pronounced thickening of the tissues. The disease is characterized by extensive airway-centered fibroelastosis of the upper lobes and bronchial abnormalities, namely bronchial wall thickening, bronchial wall deformation, and bronchiectasis. Moreover, the women presented chronic dyspnea (shortness of breath) with acute attacks of wheezing . Even with inhaled and oral corticosteroids treatment, the disease progressed in all patients leading to chronic respiratory failure. Ultimately, two of the patients underwent lung transplantation. According to a news release, the research team wrote: “All our patients had acute exacerbations of cough, dyspnea, and wheezing, which are not observed in pleuropulmonary fibroelastosis,” a recently described rare, benign disorder. Despite exhibiting characteristics similar to pleuropulmonary fibroelastosis, the patients also presented clinical, radiological, pathological, and physiological differences distinct from such conditions, researchers said. “In pleuropulmonary fibroelastosis, patients usually have dyspnea on exertion and/or cough without acute deterioration of symptoms,” the authors wrote. The team also observed that three of the five patients had an obstructive pattern
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