Allergologie select, Volume 8 (2024) - 1st issue (18 - 25)

EGPA: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) as a special presentation of chronic rhinosinusitis with nasal polyps (CRSwNP)
Jan Hagemann1,5, Martin Laudien2, Sven Becker3, Mandy Cuevas4, Felix Klimek5, Roya Kianfar5, Ingrid Casper5, Ludger Klimek5
1 Clinic for Ear, Nose and Throat Medicine, Mainz University Medical Center, Mainz, 2 Clinic for Ear, Nose and Throat Medicine, University Medicine Kiel, Kiel, 3 Clinic and Polyclinic for ENT Medicine, University Hospital Tübingen, Tübingen, 4 Clinic and Polyclinic for ENT Medicine, Carl Gustav Carus University Hospital, TU Dresden, Dresden, and 5 Center for Rhinology and Allergology, Wiesbaden, Germany

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DOI 10.5414/ALX02475E

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) was formerly known as Churg-Strauss syndrome. The condition is characterized by disseminated necrotizing vasculitis with extravascular granulomas associated with hypereosinophilia. The vasculitides affect small vessels and are associated with antineutrophil cytoplasmic antibodies (ANCAs) detectable in the blood. Distinguishing between type 2-mediated chronic airway inflammation such as chronic rhinosinusitis with nasal polyps (CRSwNP) without vasculitis can be clinically challenging and should be considered. Materials and methods: Immunological background, diagnosis, and therapy of EGPA were identified through literature searches in Medline, PubMed, as well as national and international studies (ClinicalTrials.gov) and the Cochrane Library. Human studies published up to and including 10/2023 on the topic were considered. Results: In cases of deteriorating general health with previously known eosinophilic inflammation of the upper and lower airways, EGPA and its interdisciplinary investigation should be considered. Various types of eosinophilic inflammation and syndromes must be considered differentially. Conclusion: Characterization of mucosal airway inflammation through biomarker determination is meaningful and occasionally makes the difference for targeted therapy.

Author Details

Authors

Departments

  • 1 Clinic for Ear, Nose and Throat Medicine, Mainz University Medical Center, Mainz,
  • 2 Clinic for Ear, Nose and Throat Medicine, University Medicine Kiel, Kiel,
  • 3 Clinic and Polyclinic for ENT Medicine, University Hospital Tübingen, Tübingen,
  • 4 Clinic and Polyclinic for ENT Medicine, Carl Gustav Carus University Hospital, TU Dresden, Dresden, and
  • 5 Center for Rhinology and Allergology, Wiesbaden, Germany

Address

Dr. med. Jan Hagemann, Center for Rhinology and Allergology, An den Quellen 10, 65183 Wiesbaden, Germany
Email: [email protected]

Citation

Jan Hagemann, Martin Laudien, Sven Becker, Mandy Cuevas, Felix Klimek, Roya Kianfar, Ingrid Casper, and Ludger Klimek.EGPA: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) as a special presentation of chronic rhinosinusitis with nasal polyps (CRSwNP). Allergologie Select. 2024; 8: 18-25. doi: 10.5414/ALX02475E.

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