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Airway Involvement in Granulomatosis with Polyangiitis

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Diseases of the Central Airways

Part of the book series: Respiratory Medicine ((RM))

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Abstract

The evaluation and diagnosis of a patient with suspected tracheobronchial granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis, requires a combination of clinical assessment, serologic testing, sinus and chest imaging, pulmonary function tests, bronchoscopy, and tissue biopsy. The diagnosis is established when serologic and histopathologic evidence of vasculitis and granulomatous inflammation is present in a patient with a compatible clinical presentation. In contrast to surgical lung biopsy, bronchoscopic tissue sampling of tracheobronchial luminal abnormalities only reveals histologic features of GPA in a minority of cases. The general principles of medical therapy for GPA also apply to patients who have tracheobronchial involvement. For those patients who remain symptomatic despite appropriate medical management, endoscopic management is an option.

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Authors and Affiliations

  1. Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Sonali Sethi & Kristin B. Highland

  2. Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH, USA

    Nirosshan Thiruchelvam

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  1. Sonali Sethi
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  2. Nirosshan Thiruchelvam
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  3. Kristin B. Highland
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Correspondence to Sonali Sethi .

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Editors and Affiliations

  1. Family Endowed Chair in Lung Transplanta, Lerner College of Medicine Buoncore, Clevland, Ohio, USA

    Atul C. Mehta

  2. Louis A Johnson VA Medical Center, Fairmont, West Virginia, USA

    Prasoon Jain

  3. Cleveland Clinic, Cleveland, Ohio, USA

    Thomas R. Gildea

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Sethi, S., Thiruchelvam, N., Highland, K.B. (2016). Airway Involvement in Granulomatosis with Polyangiitis. In: Mehta, A., Jain, P., Gildea, T. (eds) Diseases of the Central Airways. Respiratory Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-29830-6_4

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  • DOI: https://doi.org/10.1007/978-3-319-29830-6_4

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