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Review
. 2024 Mar 21:8:18-25.
doi: 10.5414/ALX02475E. eCollection 2024.

EGPA: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) as a special presentation of chronic rhinosinusitis with nasal polyps (CRSwNP)

Jan Hagemann  1   2 , Martin Laudien  3 , Sven Becker  4 , Mandy Cuevas  5 , Felix Klimek  2 , Roya Kianfar  2 , Ingrid Casper  2 , Ludger Klimek  2
Affiliations
Review

EGPA: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) as a special presentation of chronic rhinosinusitis with nasal polyps (CRSwNP)

Jan Hagemann et al. Allergol Select. .

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.

Keywords: ANCAs; CRSwNP; Churg-Strauss syndrome; EGPA; eosinophilic granulomatosis with polyangiitis; eosinophils; vasculitis.

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Conflict of interest statement

J. Hagemann received grants from GSK, Sanofi, HAL Allergy, Novartis Pharma GmbH for lectures and consultations, outside the submitted work. M. Laudien received funds and/or fees from Olympus Deutschland GmbH, Olympus Europa SE & CO. KG, Novartis Pharma GmbH, Sanofi-Aventis Deutschland GmbH, Brainlab Sales GmbH, CSL Vifor, GlaxoSmithKline GmbH & Co. KG, Medtronic, Fiagon AG Medical Technologies, AEDA. He is board member of John Grube Foundation and chair of AG Rhinologie/Rhinochirurgie DGHNO. M. Cuevas has received honoraria from AstraZeneca, GSK, Sanofi, and Novartis, outside the submitted work. L. Klimek has received research grants and/or lecture fees from Allergy Therapeutics/ Bencard, UK/Germany; ALK-Abelló, Denmark; Allergopharma, Germany; ASIT Biotech, Belgium; AstraZeneca, Sweden, Bionorica, Germany; Biomay, Austria, Boehringer Ingelheim, Germany, Circassia, USA; Stallergene, France; Cytos, Switzerland; Curalogic, Denmark; HAL, Netherlands; Hartington, Spain; Lofarma, Italy; MEDA/Mylan, Sweden/USA; Novartis, Switzerland, Leti, Spain; ROXALL, Germany; GlaxoSmithKline (GSK), UK; Sanofi, France outside the submitted work and/or acted in an advisory capacity for the above-mentioned pharmaceutical companies. L. Klimek is editor of Allergo Journal and Allergo Journal International and he is the current President of AeDA (Ärzteverband Deutscher Allergologen) and Vice President of EAACI. R. Kianfar and F. Klimek report no conflicts of interest in connection with the present work. Table 1.Diseases involving the respiratory tract and hypereosinophilia [1]. Adult-onset eCRSwNPEGPAN-ERDAdult-onset asthmaABPAPrevalence (asthma/CRS patients)3 – 10%0.6%5 – 10%21 – 48%2.5%M : W2 : 11 : 21 : 22 : 31 : 1Appearance at the age of...30 – 505635 – 3630 – 4625 – 55 under 12RareRareRaren.a.Rare over 60RarePossibleRarePossiblePossibleClinical peculiarityNasal polyps, loss of smellSystemic vasculitis, granulomatosisSalicylate intoleranceRapid decrease in LuFuMucus plugs (bronchial tubes)Biomarkers Blood-Eo↑↑↑↑↑↑↑↑↑↑ Serum IgE (IU/mL)Variable100 – 300VariableVariable1,000 – 10,000 FeNO↑↑↑↑↑ Serum periostin↑↑→↑↑↑↑ U LTE4↑↑↑↑↑↑ EETs/EETosis++Lumen/tissuePossible++Lumen/tissue++Lumen Charcot-Leyden crystals++Lumen/tissuePossible++Lumen/tissue++Lumen OtherCST-1, eotaxin-3, IgG4ANCA, eotaxin-3, lösIL-2RMast cell/ tile markerReversibility in LuFu for diagnosisResponse to biologicsanti-IgE+++++anti-IL-5(R)+++++++++anti-IL-4R++++++anti-TSLP(+)++eCRSwNP = eosinophilic chronic rhinosinusitis with nasal polyps; EGPA = eosinophilic polyangiitis with granulomatosis; N-ERD = non-steroidal-exacerbated respiratory disease; ABPA = allergic bronchopulmonary aspergillosis; LuFu = lung function test; FeNO = fractional expiratory NO; leukotriene E4 = LTE4; EETs/EETosis = extracellular eosinophil traps; ANCA = antineutrophil cytoplasmic antibody. Table 2.The following criteria are only to be applied in the case of histological evidence of small vessel vasculitis. Clinical criteriaObstructive airway disease+3Nasal polyps / CRSwNP+3Mononeuritis multiplex+1Laboratory and biopsy criteriaBlood eosinophilia of >1,000 cells/µL or 1 × 109/L (blood)+5Extravascular, predominantly eosinophilic inflammation (biopsy)+2cANCA + / anti-PR3 + (blood)–3Hematuria–1A score of > 6 makes EGPA likely. CRSwNP = chronic rhinosinusitis with nasal polyps; cANCA = antineutrophil cytoplasmic antibody..

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