title: Airway-Centered Fibroelastosis: A Distinct Entity.
abstract: OBJECTIVE
  To describe a new entity characterized by airway-centered fibroelastosis.
  
  
  METHODS
  We identified cases with prominent airway-centered elastosis in lung samples, and little or no pleural involvement identified through a pathologic database at a single institution over an 8-year period.
  
  
  RESULTS
  Airway-centered fibroelastosis was characterized by (1) extensive airway-centered fibroelastosis of the upper lobes on histopathology and (2) marked bronchial abnormalities with bronchial wall thickening, bronchial wall deformation, and bronchiectasis, along with progressive parenchymal retraction and predominantly subpleural upper-lobe consolidations on high-resolution CT. Pateints were five nonsmoking women aged between 38 and 56 years old. They experienced chronic dyspnea with acute attacks of wheezing and dyspnea. Moderate to severe physiological abnormalities were observed, with an obstructive pattern in three cases and a restriction in two. Despite inhaled and oral corticosteroids, the disease was progressive in all patients and evolved to chronic respiratory failure, requiring lung transplantation in two patients. Four patients had chronic asthma.
  
  
  CONCLUSIONS
  We consider airway-centered fibroelastosis to be a unique and distinct pathological entity in women that needs to be individualized, with a specific clinical, imaging, and pathological presentation. We hypothesize that airway-centered fibroelastosis may be idiopathic or asthma-associated.
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors.forename: Pauline
authors.initials: P
authors.lastname: Pradere
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors.forename: Clément
authors.initials: C
authors.lastname: Gauvain
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Département d'Anatomie Pathologique, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Danel
authors.affiliation: Service de Radiologie, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Paris, France.
authors.forename: Marie Pierre
authors.initials: MP
authors.lastname: Debray
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors.forename: Raphael
authors.initials: R
authors.lastname: Borie
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service d'explorations fonctionnelles, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Paris, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Plantier
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie et Transplantation, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Paris, France.
authors.forename: Hervé
authors.initials: H
authors.lastname: Mal
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Aubier
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France. Electronic address: bruno.crestani@bch.aphp.fr.
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
authors.affiliation: INSERM U1152, Paris, France; University Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris, Hôpital Bichat, Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Taillé
mesh_terms: D000328:Adult; D001249:Asthma / Q000150:complications / Q000503:physiopathology; D001980:Bronchi / Q000473:pathology*; D001982:Bronchial Diseases / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D001987:Bronchiectasis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D015331:Cohort Studies; D016208:Databases, Factual; D018450:Disease Progression; D004547:Elastic Tissue / Q000473:pathology*; D005260:Female; D005355:Fibrosis; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008171:Lung Diseases / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D016040:Lung Transplantation; D008875:Middle Aged; D012131:Respiratory Insufficiency / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
keywords: asthma; chronic obstructive pulmonary disease; idiopathic disease; interstitial lung disease