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

• Record Type: Journal Article
• Authors: Suppiah, Ravi; Robson, Joanna C.; Grayson, Peter C.; Ponte, Cristina; Craven, Anthea; Khalid, Sara; Judge, Andrew; Hutchings, Andrew; Merkel, Peter A.; Luqmani, Raashid A.; Watts, Richard A.
• Other Names: Gatenby Paul investigator.; Hill Catherine investigator.; Ranganathan Dwarakanathan investigator.; Kronbichler Andreas investigator.; Blockmans Daniel investigator.; Barra Lillian investigator.; Carette Simon investigator.; Pagnoux Christian investigator.; Dhindsa Navjot investigator.; Fifi‐Mah Aurore investigator.; Khalidi Nader investigator.; Liang Patrick investigator.; Milman Nataliya investigator.; Pineau Christian investigator.; Tian Xinping investigator.; Wang Guochun investigator.; Wang Tian investigator.; Zhao Ming‐hui investigator.; Tesar Vladimir investigator.; Baslund Bo investigator.; Hammam Nevin investigator.; Shahin Amira investigator.; Pirila Laura investigator.; Putaala Jukka investigator.; Hellmich Bernhard investigator.; Henes Jörg investigator.; Lamprecht Peter investigator.; Neumann Thomas investigator.; Schmidt Wolfgang investigator.; Sunderkoetter Cord investigator.; Szekanecz Zoltan investigator.; Danda Debashish investigator.; Das Siddharth investigator.; Gupta Rajiva investigator.; Rajasekhar Liza investigator.; Sharma Aman investigator.; Wagh Shrikant investigator.; Clarkson Michael investigator.; Molloy Eamonn investigator.; Salvarani Carlo investigator.; Schiavon Franco investigator.; Tombetti Enrico investigator.; Vaglio Augusto investigator.; Amano Koichi investigator.; Arimura Yoshihiro investigator.; Dobashi Hiroaki investigator.; Fujimoto Shouichi investigator.; Harigai Masayoshi investigator.; Hirano Fumio investigator.; Hirahashi Junichi investigator.; Honma Sakae investigator.; Kawakami Tamihiro investigator.; Kobayashi Shigeto investigator.; Kono Hajime investigator.; Makino Hirofumi investigator.; Matsui Kazuo investigator.; Muso Eri investigator.; Suzuki Kazuo investigator.; Ikeda Kei investigator.; Takeuchi Tsutomu investigator.; Tsukamoto Tatsuo investigator.; Uchida Shunya investigator.; Wada Takashi investigator.; Yamada Hidehiro investigator.; Yamagata Kunihiro investigator.; Yumura Wako investigator.; Lai Kan Sow investigator.; Flores‐Suarez Luis Felipe investigator.; Hinojosa Andrea investigator.; Rutgers Bram investigator.; Tak Paul‐Peter investigator.; Grainger Rebecca investigator.; Quincey Vicki investigator.; Stamp Lisa investigator.; Suppiah Ravi investigator.; Besada Emilio investigator.; Diamantopoulos Andreas investigator.; Sznajd Jan investigator.; Azevedo Elsa investigator.; Geraldes Ruth investigator.; Rodrigues Miguel investigator.; Santos Ernestina investigator.; Song Yeong‐Wook investigator.; Moiseev Sergey investigator.; Hocˇevar Alojzija investigator.; Cid Maria Cinta investigator.; Moreno Xavier Solanich investigator.; Atukorala Inoshi investigator.; Berglin Ewa investigator.; Mohammed Aladdin investigator.; Segelmark Mårten investigator.; Daikeler Thomas investigator.; Direskeneli Haner investigator.; Hatemi Gulen investigator.; Kamali Sevil investigator.; Karadag Ömer investigator.; Pehlevan Seval investigator.; Adler Matthew investigator.; Basu Neil investigator.; Bruce Iain investigator.; Chakravarty Kuntal investigator.; Dasgupta Bhaskar investigator.; Flossmann Oliver investigator.; Gendi Nagui investigator.; Hassan Alaa investigator.; Hoyles Rachel investigator.; Jayne David investigator.; Jones Colin investigator.; Klocke Rainer investigator.; Lanyon Peter investigator.; Laversuch Cathy investigator.; Luqmani Raashid investigator.; Robson Joanna investigator.; Magliano Malgorzata investigator.; Mason Justin investigator.; Maw Win Win investigator.; McInnes Iain investigator.; Mclaren John investigator.; Morgan Matthew investigator.; Morgan Ann investigator.; Mukhtyar Chetan investigator.; O'Riordan Edmond investigator.; Patel Sanjeev investigator.; Peall Adrian investigator.; Robson Joanna investigator.; Venkatachalam Srinivasan investigator.; Vermaak Erin investigator.; Menon Ajit investigator.; Watts Richard investigator.; Yee Chee‐Seng investigator.; Albert Daniel investigator.; Calabrese Leonard investigator.; Chung Sharon investigator.; Forbess Lindsy investigator.; Gaffo Angelo investigator.; Gewurz‐Singer Ora investigator.; Grayson Peter investigator.; Liang Kimberly investigator.; Matteson Eric investigator.; Merkel Peter A. investigator.; Springer Jason investigator.; Sreih Antoine investigator.;
• Is Part Of: Arthritis & rheumatology. Volume 74:Issue 3(2022)
• Page Start: 400
• Page End: 406
• Publication Date: 2022-02-02
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.41983
• Languages: English
• ISSNs: 2326-5191

Abstract

Abstract : Objective: To develop and validate classification criteria for microscopic polyangiitis (MPA). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate 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 MPA consisted of 149 cases of MPA and 408 comparators. The validation set consisted of an additional 142 cases of MPA and 414 comparators. From 91 candidate items, regression analysis identified 10 items for MPA, 6 of which were retained. The final criteria and their weights were as follows: perinuclear antineutrophil cytoplasmic antibody (ANCA) or anti–myeloperoxidase‐ANCA positivity (+6), pauci‐immune glomerulonephritis (+3), lung fibrosis or interstitial lung disease (+3), sino‐nasal symptoms or signs (−3), cytoplasmic ANCA or anti–proteinase 3 ANCA positivity (−1), and eosinophil count ≥1 × 10 9 /liter (−4). After excluding mimics of vasculitis, a patient with a diagnosis of small‐ or medium‐vessel vasculitis could be classified as having MPA with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 91% (95% confidence interval [95% CI] 85–95%) and the specificity was 94% (95% CI 92–96%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research.