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2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis

Grayson, Peter C.; Luqmani, Raashid A.; Merkel, Peter A.; Gatenby, Paul; Watts, Richard A.; Hill, Catherine; Ranganathan, Dwarakanathan; Kronbichler, Andreas; Blockmans, Daniel; Barra, Lillian; Carette, Simon; Pagnoux, Christian; Dhindsa, Navjot; Fifi-Mah, Aurore; Khalidi, Nader; Liang, Patrick; Milman, Nataliya; Pineau, Christian; Tian, Xinping; Wang, Guochun; Wang, Tian; Zhao, Ming hui; Tesar, Vladimir; Baslund, Bo; Hammam, Nevin; Shahin, Amira; Pirila, Laura; Putaala, Jukka; Hellmich, Bernhard; Henes, J?rg; Lamprecht, Peter; Neumann, Thomas; Schmidt, Wolfgang; Sunderkoetter, Cord; Szekanecz, Zoltan; Danda, Debashish; Das, Siddharth; Gupta, Rajiva; Rajasekhar, Liza; Dobashi, Hiroaki; Sharma, Aman; Wagh, Shrikant; Clarkson, Michael; Kawakami, Tamihiro; Kobayashi, Shigeto; Kono, Hajime; Makino, Hirofumi; Matsui, Kazuo; Muso, Eri; Suzuki, Kazuo; Ikeda, Kei; Takeuchi, Tsutomu; Tsukamoto, Tatsuo; Uchida, Shunya; Wada, Takashi; Yamada, Hidehiro; Yamagata, Kunihiro; Yumura, Wako; Lai, Kan S...

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis Thumbnail


Authors

Peter C. Grayson

Raashid A. Luqmani

Peter A. Merkel

Paul Gatenby

Richard A. Watts

Catherine Hill

Dwarakanathan Ranganathan

Andreas Kronbichler

Daniel Blockmans

Lillian Barra

Simon Carette

Christian Pagnoux

Navjot Dhindsa

Aurore Fifi-Mah

Nader Khalidi

Patrick Liang

Nataliya Milman

Christian Pineau

Xinping Tian

Guochun Wang

Tian Wang

Ming hui Zhao

Vladimir Tesar

Bo Baslund

Nevin Hammam

Amira Shahin

Laura Pirila

Jukka Putaala

Bernhard Hellmich

J�rg Henes

Peter Lamprecht

Thomas Neumann

Wolfgang Schmidt

Cord Sunderkoetter

Zoltan Szekanecz

Debashish Danda

Siddharth Das

Rajiva Gupta

Liza Rajasekhar

Hiroaki Dobashi

Aman Sharma

Shrikant Wagh

Michael Clarkson

Tamihiro Kawakami

Shigeto Kobayashi

Hajime Kono

Hirofumi Makino

Kazuo Matsui

Eri Muso

Kazuo Suzuki

Kei Ikeda

Tsutomu Takeuchi

Tatsuo Tsukamoto

Shunya Uchida

Takashi Wada

Hidehiro Yamada

Kunihiro Yamagata

Wako Yumura

Kan Sow Lai

Luis Felipe Flores-Suarez

Andrea Hinojosa

Bram Rutgers

Paul Peter Tak

Rebecca Grainger

Vicki Quincey

Lisa Stamp

Emilio Besada

Andreas Diamantopoulos

Jan Sznajd

Elsa Azevedo

Ruth Geraldes

Miguel Rodrigues

Ernestina Santos

Yeong Wook Song

Sergey Moiseev

Alojzija Hoc?evar

Yoshihiro Arimura

Maria Cinta Cid

Xavier Solanich Moreno

Inoshi Atukorala

Ewa Berglin

Eamonn Molloy

Carlo Salvarani

Franco Schiavon

Enrico Tombetti

Augusto Vaglio

Koichi Amano

Shouichi Fujimoto

Masayoshi Harigai

Fumio Hirano

Junichi Hirahashi

Sakae Honma

Peter Grayson

Cristina Ponte

Ravi Suppiah

Jo Robson Jo.Robson@uwe.ac.uk
Consultant Associate Professor in Rheumatology

Anthea Craven

Andrew Judge

Sara Khalid

Andrew Hutchings

Raashid Luqmani

Richard Watts

Peter Merkel



Abstract

Objective: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. Results: The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1 × 109/liter (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti–proteinase 3 ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1), and hematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% confidence interval [95% CI] 77–91%) and the specificity was 99% (95% CI 98–100%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for EGPA demonstrate strong performance characteristics and are validated for use in research.

Citation

Honma, S., Schiavon, F., Salvarani, C., Molloy, E., Berglin, E., Moreno, X. S., …Merkel, P. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 386-392. https://doi.org/10.1002/art.41982

Journal Article Type Article
Acceptance Date Apr 16, 2021
Online Publication Date Feb 2, 2022
Publication Date Mar 1, 2022
Deposit Date Jun 2, 2021
Publicly Available Date Mar 29, 2024
Journal Arthritis and Rheumatology
Print ISSN 2326-5191
Electronic ISSN 2326-5205
Publisher Wiley
Peer Reviewed Peer Reviewed
Volume 74
Issue 3
Pages 386-392
DOI https://doi.org/10.1002/art.41982
Public URL https://uwe-repository.worktribe.com/output/7438057

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This is the peer reviewed version of the following article: This is the peer reviewed version of the following article: Honma, S., Schiavon, F., Salvarani, C., Molloy, E., Berglin, E., Moreno, X. S., …Merkel, P. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 386-392, which has been published in final form at https://doi.org/10.1002/art.41982. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited, which has been published in final form at https://doi.org/10.1002/art.41982. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited.



2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis (418 Kb)
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http://www.rioxx.net/licenses/all-rights-reserved

Publisher Licence URL
http://www.rioxx.net/licenses/all-rights-reserved

Copyright Statement
This is the peer reviewed version of the following article: Honma, S., Schiavon, F., Salvarani, C., Molloy, E., Berglin, E., Moreno, X. S., …Merkel, P. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Arthritis and Rheumatology, 74(3), 386-392, which has been published in final form at https://doi.org/10.1002/art.41982. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited.




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