Ravi Suppiah1, Joanna C Robson2, Peter C Grayson3, Cristina Ponte4, Anthea Craven5, Sara Khalid5, Andrew Judge6, Andrew Hutchings7, Peter A Merkel8, Raashid A Luqmani5, Richard A Watts9. 1. Auckland District Health Board, Auckland, New Zealand. 2. Centre for Health and Clinical Research, University of the West of England, and University Hospitals and Weston NHS Foundation Trust, Bristol, UK. 3. National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. 4. Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Universidade de Lisboa, and Centro Académico de Medicina de Lisboa, Lisbon, Portugal. 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. University of Pennsylvania, Philadelphia. 9. University of East Anglia, Norwich, UK.
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 × 109 /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.
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 × 109 /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.
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