Joanna C Robson1, Peter C Grayson2, Cristina Ponte3, Ravi Suppiah4, Anthea Craven5, Andrew Judge6, Sara Khalid5, Andrew Hutchings7, Richard A Watts8, Peter A Merkel9, Raashid A Luqmani5. 1. Centre for Health and Clinical Research, University of the West of England, and University Hospitals and Weston NHS Foundation Trust, Bristol, UK. 2. National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. 3. Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Universidade de Lisboa, and Centro Académico de Medicina de Lisboa, Lisbon, Portugal. 4. Auckland District Health Board, Auckland, New Zealand. 5. Oxford NIHR Biomedical Research Centre and University of Oxford, Oxford, UK. 6. Oxford NIHR Biomedical Research Centre and University of Oxford, Oxford, UK, and Bristol NIHR Biomedical Research Centre and University of Bristol, Bristol, UK. 7. London School of Hygiene and Tropical Medicine, London, UK. 8. Oxford NIHR Biomedical Research Centre and University of Oxford, Oxford, UK, and University of East Anglia, Norwich, UK. 9. University of Pennsylvania, Philadelphia.
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.
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.
Authors: Giorgio Trivioli; Ana Marquez; Davide Martorana; Michelangelo Tesi; Andreas Kronbichler; Paul A Lyons; Augusto Vaglio Journal: Nat Rev Rheumatol Date: 2022-09-15 Impact factor: 32.286
Authors: Joshua Zarbl; Ekaterina Eimer; Camilla Gigg; Gerlinde Bendzuck; Marianne Korinth; Corinna Elling-Audersch; Arnd Kleyer; David Simon; Sebastian Boeltz; Martin Krusche; Johanna Mucke; Felix Muehlensiepen; Nicolas Vuillerme; Gerhard Krönke; Georg Schett; Johannes Knitza Journal: RMD Open Date: 2022-09