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Orphan Tracheopathies

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Abstract

Tracheal diseases are most commonly infectious or neoplastic in nature. However, there are several uncommon tracheal disorders that can be easily overlooked in patients presenting with nonspecific respiratory complaints. These tracheopathies include idiopathic subglottic stenosis (ISS), tracheobronchopathia osteochondroplastica (TPO), tracheomalacia (Mounier-Kuhn syndrome), tracheobronchomegaly, and tracheopathies associated with systemic diseases including relapsing polychondritis, granulomatosis with polyangiitis (GPA), amyloidosis, and sarcoidosis.

A diagnostic evaluation includes computed tomography (CT) scanning and bronchoscopy combined with assessment of the clinical context. Treatment of tracheopathy is determined by the underlying cause and the characteristics of the tracheal lesion including its location, extent, and severity of airway compromise. Bronchoscopic interventions including laser therapy and stent placement can benefit patients with stenotic tracheal lesions as well as those with collapsible airways. In those patients with tracheopathy associated with systemic disorders, the underlying disease needs to be addressed.

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Author information

Authors and Affiliations

  1. Division of Allergy, Pulmonary and Critical Care, Vanderbilt University, Nashville, TN, USA

    Fabien Maldonado

  2. Department of Diseases of the Thorax, Interventional Pulmonology Unit, GB Morgagni Hospital, Forli, Italy

    Sara Tomassetti

  3. Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA

    Jay H. Ryu

Authors
  1. Fabien Maldonado
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  2. Sara Tomassetti
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  3. Jay H. Ryu
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Corresponding author

Correspondence to Jay H. Ryu .

Editor information

Editors and Affiliations

  1. Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases (OrphaLung), Louis Pradel Hospital, University of Lyon, Lyon, France

    Vincent Cottin

  2. Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy

    Luca Richeldi

  3. National Jewish Health, Denver, CO, USA

    Kevin Brown

  4. Division of Pulmonary, Critical Care, and Sleep Medicine Department of Internal Medicine, University of Cincinnati, Cincinnati, OH, USA

    Francis X. McCormack

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Maldonado, F., Tomassetti, S., Ryu, J.H. (2023). Orphan Tracheopathies. In: Cottin, V., Richeldi, L., Brown, K., McCormack, F.X. (eds) Orphan Lung Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-12950-6_5

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