2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Issue 3 (2nd February 2022)

• Record Type: Journal Article
• Authors: Grayson, Peter C; Ponte, Cristina; Suppiah, Ravi; Robson, Joanna C; Craven, Anthea; Judge, Andrew; Khalid, Sara; Hutchings, Andrew; Luqmani, Raashid A; Watts, Richard A; Merkel, Peter A
• Other Names: author non-byline.; Gatenby Paul author non-byline.; Hill Catherine author non-byline.; Ranganathan Dwarakanathan author non-byline.; Kronbichler Andreas author non-byline.; Blockmans Daniel author non-byline.; Barra Lillian author non-byline.; Carette Simon author non-byline.; Pagnoux Christian author non-byline.; Dhindsa Navjot author non-byline.; Fifi-Mah Aurore author non-byline.; Khalidi Nader author non-byline.; Liang Patrick author non-byline.; Milman Nataliya author non-byline.; Pineau Christian author non-byline.; Tian Xinping author non-byline.; Wang Guochun author non-byline.; Wang Tian author non-byline.; Zhao Ming-hui author non-byline.; Tesar Vladimir author non-byline.; Baslund Bo author non-byline.; Hammam Nevin author non-byline.; Shahin Amira author non-byline.; Pirila Laura author non-byline.; Putaala Jukka author non-byline.; Hellmich Bernhard author non-byline.; Henes Jörg author non-byline.; Lamprecht Peter author non-byline.; Neumann Thomas author non-byline.; Schmidt Wolfgang author non-byline.; Sunderkoetter Cord author non-byline.; Szekanecz Zoltan author non-byline.; Danda Debashish author non-byline.; Das Siddharth author non-byline.; Gupta Rajiva author non-byline.; Rajasekhar Liza author non-byline.; Sharma Aman author non-byline.; Wagh Shrikant author non-byline.; Clarkson Michael author non-byline.; Molloy Eamonn author non-byline.; Salvarani Carlo author non-byline.; Schiavon Franco author non-byline.; Tombetti Enrico author non-byline.; Vaglio Augusto author non-byline.; Amano Koichi author non-byline.; Arimura Yoshihiro author non-byline.; Dobashi Hiroaki author non-byline.; Fujimoto Shouichi author non-byline.; Harigai Masayoshi author non-byline.; Hirano Fumio author non-byline.; Hirahashi Junichi author non-byline.; Honma Sakae author non-byline.; Kawakami Tamihiro author non-byline.; Kobayashi Shigeto author non-byline.; Kono Hajime author non-byline.; Makino Hirofumi author non-byline.; Matsui Kazuo author non-byline.; Muso Eri author non-byline.; Suzuki Kazuo author non-byline.; Ikeda Kei author non-byline.; Takeuchi Tsutomu author non-byline.; Tsukamoto Tatsuo author non-byline.; Uchida Shunya author non-byline.; Wada Takashi author non-byline.; Yamada Hidehiro author non-byline.; Yamagata Kunihiro author non-byline.; Yumura Wako author non-byline.; Lai Kan Sow author non-byline.; Flores-Suarez Luis Felipe author non-byline.; Hinojosa Andrea author non-byline.; Rutgers Bram author non-byline.; Tak Paul-Peter author non-byline.; Grainger Rebecca author non-byline.; Quincey Vicki author non-byline.; Stamp Lisa author non-byline.; Suppiah Ravi author non-byline.; Besada Emilio author non-byline.; Diamantopoulos Andreas author non-byline.; Sznajd Jan author non-byline.; Azevedo Elsa author non-byline.; Geraldes Ruth author non-byline.; Rodrigues Miguel author non-byline.; Santos Ernestina author non-byline.; Song Yeong-Wook author non-byline.; Moiseev Sergey author non-byline.; Hocˇevar Alojzija author non-byline.; Cid Maria Cinta author non-byline.; Moreno Xavier Solanich author non-byline.; Atukorala Inoshi author non-byline.; Berglin Ewa author non-byline.; Mohammed Aladdin author non-byline.; Segelmark Mårten author non-byline.; Daikeler Thomas author non-byline.; Direskeneli Haner author non-byline.; Hatemi Gulen author non-byline.; Kamali Sevil author non-byline.; Karadag Ömer author non-byline.; Pehlevan Seval author non-byline.; Adler Matthew author non-byline.; Basu Neil author non-byline.; Bruce Iain author non-byline.; Chakravarty Kuntal author non-byline.; Dasgupta Bhaskar author non-byline.; Flossmann Oliver author non-byline.; Gendi Nagui author non-byline.; Hassan Alaa author non-byline.; Hoyles Rachel author non-byline.; Jayne David author non-byline.; Jones Colin author non-byline.; Klocke Rainer author non-byline.; Lanyon Peter author non-byline.; Laversuch Cathy author non-byline.; Luqmani Raashid author non-byline.; Robson Joanna author non-byline.; Magliano Malgorzata author non-byline.; Mason Justin author non-byline.; Maw Win Win author non-byline.; McInnes Iain author non-byline.; Mclaren John author non-byline.; Morgan Matthew author non-byline.; Morgan Ann author non-byline.; Mukhtyar Chetan author non-byline.; O'Riordan Edmond author non-byline.; Patel Sanjeev author non-byline.; Peall Adrian author non-byline.; Robson Joanna author non-byline.; Venkatachalam Srinivasan author non-byline.; Vermaak Erin author non-byline.; Menon Ajit author non-byline.; Watts Richard author non-byline.; Yee Chee-Seng author non-byline.; Albert Daniel author non-byline.; Calabrese Leonard author non-byline.; Chung Sharon author non-byline.; Forbess Lindsy author non-byline.; Gaffo Angelo author non-byline.; Gewurz-Singer Ora author non-byline.; Grayson Peter author non-byline.; Liang Kimberly author non-byline.; Matteson Eric author non-byline.; Merkel Peter A. author non-byline.; Springer Jason author non-byline.; Sreih Antoine author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 81:Issue 3(2022)
• Page Start: 309
• Page End: 314
• Publication Date: 2022-02-02
• Subjects: autoimmune diseases -- rehabilitation -- arthritis; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2021-221794
• Languages: English
• ISSNs: 0003-4967

Abstract

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 five 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 ≥1×10 9 /L (+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 haematuria (−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% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.