2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis

Abstract

ObjectiveTo develop and validate classification criteria for microscopic polyangiitis (MPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five 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.ResultsThe 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/L (−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% CI 85% to 95%) and the specificity was 94% (95% CI 92% to 96%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research.

The Complement System and ANCA Associated Vasculitis in the Era of Anti-Complement Drugs

“…The diagnosis of AAV is often based on the European Medicines Agency (EMA) classification criteria written by Watts ( 5 ), but new classification criteria for each AAV have been proposed by ACR/EULAR in 2022 ( 6 – 8 ).…”

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

“…Antineutrophil cytoplasmic antibodies (ANCA) are generally detected in multisystemic diseases, namely ANCA-associated vasculitides such as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) [1][2][3]. The presence of a lung lesion is a common and important clinical feature in ANCA-associated vasculitides [2,4]. However, interstitial lung disease (ILD) is rarely seen in patients with GPA and EGPA [1][2][3][4], but the presence of ILD is a common and important clinical feature in MPA [1,3,4].…”

“…The presence of a lung lesion is a common and important clinical feature in ANCA-associated vasculitides [2,4]. However, interstitial lung disease (ILD) is rarely seen in patients with GPA and EGPA [1][2][3][4], but the presence of ILD is a common and important clinical feature in MPA [1,3,4]. In addition, MPO-ANCA have been frequently identified in patients with ILD without multisystemic disease.…”

Response to: Correspondence on ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis’ by Joanna C Robsonet aland ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis’ by Pimentel-Quirozet al

“…We read the comments by Pimentel-Quiroz et al with interest1 and agree it is important to test the performance characteristics of the 2022 ACR/EULAR Classification Criteria for the ANCA-associated vasculitides (AAV) in different populations around the world 2–4. These efforts are needed to understand whether regional differences in disease expression and clinical evaluation impact the utility of the new criteria in specific populations.…”