Objective: To develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA). 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 GPA consisted of 578 cases of GPA and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The final criteria and their weights were as follows: bloody nasal discharge, nasal crusting, or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti–proteinase 3 ANCA positivity (+5); pulmonary nodules, mass, or cavitation on chest imaging (+2); granuloma or giant cells on biopsy (+2); inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); pauci-immune glomerulonephritis (+1); perinuclear ANCA or antimyeloperoxidase ANCA positivity (−1); and eosinophil count ≥1 × 109/liter (−4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score was ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 93% (95% confidence interval [95% CI] 87–96%) and the specificity was 94% (95% CI 89–97%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for GPA demonstrate strong performance characteristics and are validated for use in research.
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis / Robson, Joanna C.; Grayson, Peter C.; Ponte, Cristina; Suppiah, Ravi; Craven, Anthea; Judge, Andrew; Khalid, Sara; Hutchings, Andrew; Watts, Richard A.; Merkel, Peter A.; Luqmani, Raashid A.; DCVAS Study Group: Paul Gatenby, 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, Jörg Henes, Peter Lamprecht, Thomas Neumann, Wolfgang Schmidt, Cord Sunderkoetter, Zoltan Szekanecz, Debashish Danda, Siddharth Das, Rajiva Gupta, Liza Rajasekhar, Aman Sharma, Shrikant Wagh, Michael Clarkson, Eamonn Molloy, Carlo Salvarani, Franco Schiavon, Enrico Tombetti, Augusto Vaglio, Koichi Amano, Yoshihiro Arimura, Hiroaki Dobashi, Shouichi Fujimoto, Masayoshi Harigai, Fumio Hirano, Junichi Hirahashi, Sakae Honma, 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, Ravi Suppiah, Emilio Besada, Andreas Diamantopoulos, Jan Sznajd, Elsa Azevedo, Ruth Geraldes, Miguel Rodrigues, Ernestina Santos, Yeong-Wook Song, Sergey Moiseev, Alojzija Hocˇevar, Maria Cinta Cid, Xavier Solanich Moreno, Inoshi Atukorala, Ewa Berglin, Aladdin Mohammed, Mårten Segelmark, Thomas Daikeler, Haner Direskeneli, Gulen Hatemi, Sevil Kamali, Ömer Karadag, Seval Pehlevan, Matthew Adler, Neil Basu, Iain Bruce, Kuntal Chakravarty, Bhaskar Dasgupta, Oliver Flossmann, Nagui Gendi, Alaa Hassan, Rachel Hoyles, David Jayne, Colin Jones, Rainer Klocke, Peter Lanyon, Cathy Laversuch, Raashid Luqmani, Joanna Robson, Malgorzata Magliano, Justin Mason, Win Win Maw, Iain McInnes, John Mclaren, Matthew Morgan, Ann Morgan, Chetan Mukhtyar, Edmond O'Riordan, Sanjeev Patel, Adrian Peall, Joanna Robson, Srinivasan Venkatachalam, Erin Vermaak, Ajit Menon, Richard Watts, Chee-Seng Yee, Daniel Albert, Leonard Calabrese, Sharon Chung, Lindsy Forbess, Angelo Gaffo, Ora Gewurz-Singer, Peter Grayson, Kimberly Liang, Eric Matteson, Peter A Merkel, Jason Springer, Antoine Sreih. - In: ARTHRITIS & RHEUMATOLOGY. - ISSN 2326-5191. - ELETTRONICO. - 74:(2022), pp. 393-399. [10.1002/art.41986]
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis
Augusto Vaglio;
2022
Abstract
Objective: To develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA). 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 GPA consisted of 578 cases of GPA and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The final criteria and their weights were as follows: bloody nasal discharge, nasal crusting, or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti–proteinase 3 ANCA positivity (+5); pulmonary nodules, mass, or cavitation on chest imaging (+2); granuloma or giant cells on biopsy (+2); inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); pauci-immune glomerulonephritis (+1); perinuclear ANCA or antimyeloperoxidase ANCA positivity (−1); and eosinophil count ≥1 × 109/liter (−4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score was ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 93% (95% confidence interval [95% CI] 87–96%) and the specificity was 94% (95% CI 89–97%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for GPA demonstrate strong performance characteristics and are validated for use in research.
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