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Granulomatosis with Polyangiitis

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Airway diseases

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Abstract

Granulomatosis with polyangiitis (GPA) is a form of systemic vasculitis that affects small- and medium-sized blood vessels. It is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis. It was first described in 1937 by German pathologist Friedrich Wegener, and till 2011, the disease was called Wegener’s granulomatosis. In January 2011, the Board of Directors of the American College of Rheumatology (ACR), the American Society of Nephrology (ASN), and the European League Against Rheumatism (EULAR) changed the name to disease-descriptive nomenclature, and since 2011, it has been called GPA. Also, the term “limited” GPA was used to describe the isolated disease to the upper respiratory tract and lungs. However, during the disease, systemic involvement was subsequently developed in many patients, and therefore the use of the term “limited” was also left due to its confusing terminology [1–5].

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Author information

Authors and Affiliations

  1. Department of Respiratory Medicine, School of Medicine, Recep Tayyip Erdogan University, Rize, Turkey

    Dilek Karadoğan

  2. Department of Chest Diseases, School of Medicine, University Lokman Hekim University, Ankara, Turkey

    Adile Berna Dursun

  3. Medical University of Silesia, School of Medicine in Katowice, Department of Internal Diseases, Rheumatology and Clinical Immunology, Katowice, Poland

    Robert Pieczyrak

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  1. Dilek Karadoğan
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  2. Adile Berna Dursun
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  3. Robert Pieczyrak
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Editor information

Editors and Affiliations

  1. Department of Otorhinolaryngology, Eskişehir Osmangazi University, Eskisehir, Türkiye

    Cemal Cingi

  2. Department of Pulmonology, Celal Bayar University, Manisa, Türkiye

    Arzu Yorgancıoğlu

  3. Department of Otorhinolaryngology, Kırıkkale University, Kırıkkale, Türkiye

    Nuray Bayar Muluk

  4. Federal University of Bahia School of Medicine and ProAR Foundation, Salvador - Bahia, Brazil

    Alvaro A. Cruz

Section Editor information

  1. Department of Pulmonology, Gazi University School of Medicine, Ankara, Turkey

    I. K. Oguzulgen MD

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Karadoğan, D., Dursun, A.B., Pieczyrak, R. (2023). Granulomatosis with Polyangiitis. In: Cingi, C., Yorgancıoğlu, A., Bayar Muluk, N., Cruz, A.A. (eds) Airway diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-22483-6_90-1

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  • DOI: https://doi.org/10.1007/978-3-031-22483-6_90-1

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  • Print ISBN: 978-3-031-22482-9

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