2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis

Grayson, P.C.; Ponte, C.; Suppiah, R.; Robson, J.C.; Craven, A.; Judge, A.; Khalid, S.; Hutchings, A.; Luqmani, R.A.; Watts, R.A.; Merkel, P.A.; Gatenby, P.; Hill, C.; Ranganathan, D.; Kronbichler, A.; Blockmans, D.; Barra, L.; Carette, S.; Pagnoux, C.; Dhindsa, N.; Fifi-Mah, A.; Khalidi, N.; Liang, P.; Milman, N.; Pineau, C.; Tian, X.; Wang, G.; Wang, T.; Zhao, M.-.; Tesar, V.; Baslund, B.; Hammam, N.; Shahin, A.; Pirila, L.; Putaala, J.; Hellmich, B.; Henes, J.; Lamprecht, P.; Neumann, T.; Schmidt, W.; Sunderkoetter, C.; Szekanecz, Z.; Danda, D.; Das, S.; Gupta, R.; Rajasekhar, L.; Sharma, A.; Wagh, S.; Clarkson, M.; Molloy, E.; Salvarani, C.; Schiavon, F.; Tombetti, E.; Vaglio, A.; Amano, K.; Arimura, Y.; Dobashi, H.; Fujimoto, S.; Harigai, M.; Hirano, F.; Hirahashi, J.; Honma, S.; Kawakami, T.; Kobayashi, S.; Kono, H.; Makino, H.; Matsui, K.; Muso, E.; Suzuki, K.; Ikeda, K.; Takeuchi, T.; Tsukamoto, T.; Uchida, S.; Wada, T.; Yamada, H.; Yamagata, K.; Yumura, W.; Lai, K.S.; Flores-Suarez, L.F.; Hinojosa, A.; Rutgers, B.; Tak, P.-.; Grainger, R.; Quincey, V.; Stamp, L.; Suppiah, R.; Besada, E.; Diamantopoulos, A.; Sznajd, J.; Azevedo, E.; Geraldes, R.; Rodrigues, M.; Santos, E.; Song, Y.-.; Moiseev, S.; Hocevar, A.; Cid, M.C.; Moreno, X.S.; Atukorala, I.; Berglin, E.; Mohammed, A.; Segelmark, M.; Daikeler, T.; Direskeneli, H.; Hatemi, G.; Kamali, S.; Karadag, O.; Pehlevan, S.; Adler, M.; Basu, N.; Bruce, I.; Chakravarty, K.; Dasgupta, B.; Flossmann, O.; Gendi, N.; Hassan, A.; Hoyles, R.; Jayne, D.; Jones, C.; Klocke, R.; Lanyon, P.; Laversuch, C.; Luqmani, R.; Robson, J.; Magliano, M.; Mason, J.; Maw, W.W.; Mcinnes, I.; Mclaren, J.; Morgan, M.; Morgan, A.; Mukhtyar, C.; O'Riordan, E.; Patel, S.; Peall, A.; Robson, J.; Venkatachalam, S.; Vermaak, E.; Menon, A.; Watts, R.; Yee, C.-.; Albert, D.; Calabrese, L.; Chung, S.; Forbess, L.; Gaffo, A.; Gewurz-Singer, O.; Grayson, P.; Liang, K.; Matteson, E.; Merkel, P.A.; Springer, J.; Sreih, A.

doi:10.1002/art.41982

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.

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis / P.C. Grayson, C. Ponte, R. Suppiah, J.C. Robson, A. Craven, A. Judge, S. Khalid, A. Hutchings, R.A. Luqmani, R.A. Watts, P.A. Merkel, P. Gatenby, C. Hill, D. Ranganathan, A. Kronbichler, D. Blockmans, L. Barra, S. Carette, C. Pagnoux, N. Dhindsa, A. Fifi-Mah, N. Khalidi, P. Liang, N. Milman, C. Pineau, X. Tian, G. Wang, T. Wang, M.-. Zhao, V. Tesar, B. Baslund, N. Hammam, A. Shahin, L. Pirila, J. Putaala, B. Hellmich, J. Henes, P. Lamprecht, T. Neumann, W. Schmidt, C. Sunderkoetter, Z. Szekanecz, D. Danda, S. Das, R. Gupta, L. Rajasekhar, A. Sharma, S. Wagh, M. Clarkson, E. Molloy, C. Salvarani, F. Schiavon, E. Tombetti, A. Vaglio, K. Amano, Y. Arimura, H. Dobashi, S. Fujimoto, M. Harigai, F. Hirano, J. Hirahashi, S. Honma, T. Kawakami, S. Kobayashi, H. Kono, H. Makino, K. Matsui, E. Muso, K. Suzuki, K. Ikeda, T. Takeuchi, T. Tsukamoto, S. Uchida, T. Wada, H. Yamada, K. Yamagata, W. Yumura, K.S. Lai, L.F. Flores-Suarez, A. Hinojosa, B. Rutgers, P.-. Tak, R. Grainger, V. Quincey, L. Stamp, R. Suppiah, E. Besada, A. Diamantopoulos, J. Sznajd, E. Azevedo, R. Geraldes, M. Rodrigues, E. Santos, Y.-. Song, S. Moiseev, A. Hocevar, M.C. Cid, X.S. Moreno, I. Atukorala, E. Berglin, A. Mohammed, M. Segelmark, T. Daikeler, H. Direskeneli, G. Hatemi, S. Kamali, O. Karadag, S. Pehlevan, M. Adler, N. Basu, I. Bruce, K. Chakravarty, B. Dasgupta, O. Flossmann, N. Gendi, A. Hassan, R. Hoyles, D. Jayne, C. Jones, R. Klocke, P. Lanyon, C. Laversuch, R. Luqmani, J. Robson, M. Magliano, J. Mason, W.W. Maw, I. Mcinnes, J. Mclaren, M. Morgan, A. Morgan, C. Mukhtyar, E. O'Riordan, S. Patel, A. Peall, J. Robson, S. Venkatachalam, E. Vermaak, A. Menon, R. Watts, C.-. Yee, D. Albert, L. Calabrese, S. Chung, L. Forbess, A. Gaffo, O. Gewurz-Singer, P. Grayson, K. Liang, E. Matteson, P.A. Merkel, J. Springer, A. Sreih. - In: ARTHRITIS & RHEUMATOLOGY. - ISSN 2326-5191. - 74:3(2022), pp. 386-392. [10.1002/art.41982]

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis

Grayson P. C.;Ponte C.;Suppiah R.;Robson J. C.;Craven A.;Judge A.;Khalid S.;Hutchings A.;Luqmani R. A.;Watts R. A.;Merkel P. A.;Gatenby P.;Hill C.;Ranganathan D.;Kronbichler A.;Blockmans D.;Barra L.;Carette S.;Pagnoux C.;Dhindsa N.;Fifi-Mah A.;Khalidi N.;Liang P.;Milman N.;Pineau C.;Tian X.;Wang G.;Wang T.;Zhao M. -H.;Tesar V.;Baslund B.;Hammam N.;Shahin A.;Pirila L.;Putaala J.;Hellmich B.;Henes J.;Lamprecht P.;Neumann T.;Schmidt W.;Sunderkoetter C.;Szekanecz Z.;Danda D.;Das S.;Gupta R.;Rajasekhar L.;Sharma A.;Wagh S.;Clarkson M.;Molloy E.;Salvarani C.;Schiavon F.;E. Tombetti;Vaglio A.;Amano K.;Arimura Y.;Dobashi H.;Fujimoto S.;Harigai M.;Hirano F.;Hirahashi J.;Honma S.;Kawakami T.;Kobayashi S.;Kono H.;Makino H.;Matsui K.;Muso E.;Suzuki K.;Ikeda K.;Takeuchi T.;Tsukamoto T.;Uchida S.;Wada T.;Yamada H.;Yamagata K.;Yumura W.;Lai K. S.;Flores-Suarez L. F.;Hinojosa A.;Rutgers B.;Tak P. -P.;Grainger R.;Quincey V.;Stamp L.;Suppiah R.;Besada E.;Diamantopoulos A.;Sznajd J.;Azevedo E.;Geraldes R.;Rodrigues M.;Santos E.;Song Y. -W.;Moiseev S.;Hocevar A.;Cid M. C.;Moreno X. S.;Atukorala I.;Berglin E.;Mohammed A.;Segelmark M.;Daikeler T.;Direskeneli H.;Hatemi G.;Kamali S.;Karadag O.;Pehlevan S.;Adler M.;Basu N.;Bruce I.;Chakravarty K.;Dasgupta B.;Flossmann O.;Gendi N.;Hassan A.;Hoyles R.;Jayne D.;Jones C.;Klocke R.;Lanyon P.;Laversuch C.;Luqmani R.;Robson J.;Magliano M.;Mason J.;Maw W. W.;McInnes I.;Mclaren J.;Morgan M.;Morgan A.;Mukhtyar C.;O'Riordan E.;Patel S.;Peall A.;Robson J.;Venkatachalam S.;Vermaak E.;Menon A.;Watts R.;Yee C. -S.;Albert D.;Calabrese L.;Chung S.;Forbess L.;Gaffo A.;Gewurz-Singer O.;Grayson P.;Liang K.;Matteson E.;Merkel P. A.;Springer J.;Sreih A.

2022

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.

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	Settori scientifico-disciplinari dell'articolo (validi dal 09/05/2024)
	
				Settore MEDS-09/C - Reumatologia
Settore MEDS-05/A - Medicina interna
			
	Titolo del progetto
	
	Titolo Progetto
	
									MICA: Treatment According to Response in Giant cEll arTeritis (TARGET)
								
	Nome finanziatore
	
										UK Research and Innovation
									
	Finanziamento
	
									MRC
								
	N. Contratto
	
									MR/N011775/1
								
	Data di pubblicazione
	
				2022
			
	Rivista in ANCE
	
				ARTHRITIS & RHEUMATOLOGY
			
	DOI
	
				https://dx.doi.org/10.1002/art.41982
			
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				Article (author)
			
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				01 - Articolo su periodico

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