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

Abstract

ObjectiveTo develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five 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.ResultsThe 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 /L (−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% CI 87% to 96%) and the specificity was 94% (95% CI 89% to 97%).ConclusionThe 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.

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.…”

New Classification Criteria for Small‐Vessel Vasculitis: Is Antineutrophil Cytoplasmic Antibody Inclusion Their Major Advance?

“…In this issue of Arthritis & Rheumatology , the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria for granulomatosis with polyangiitis (GPA) (4), microscopic polyangiitis (MPA) (5), and eosinophilic granulomatosis with polyangiitis (EGPA) (6) are presented. They are based on the analysis of the most ambitious prospective cohort study ever on vasculitis, the Diagnostic and Classification Criteria in Vasculitis (DCVAS) project, involving almost 7,000 patients from 136 sites worldwide.…”

“…As a consequence, the criteria of ANCA detection and type outweighed the other (diagnostically relevant) typical characteristics of small‐vessel vasculitis, such as certain clinical manifestations (e.g., constitutional symptoms or skin lesions) or histologically confirmed pauci‐immune glomerulonephritis, which are either not included among the new classification criteria or were accorded relatively low weights. Patients with ANCA‐negative limited GPA, for example, would hardly fulfill the new classification criteria and therefore be missed if the criteria were (mis)used for diagnostic purposes (4).…”

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’

“…The new classification criteria for antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) have been eagerly anticipated since the publication of the provisional criteria in 20171 and thus they are quite welcomed 2 3. Limitations of the previous criteria used for these conditions include the low sensitivity of the 1990 American College of Rheumatology (ACR) classification criteria, the fact that the patient’s ANCA status had not been included, that there were no previous classification criteria for microscopic polyangiitis (MPA), and the fact that the number of criteria rather than a weighted criteria were used.…”

“…Given these considerations, we read with great interest the articles on the new classification criteria for GPA and MPA by Robson et al and Suppiah et al , respectively,2 3 which have shown to have good sensitivity and specificity. These criteria have been validated by the Diagnostic and Classification Criteria in Vasculitis (DCVAS) project which recruited 6991 participants (among patients with AAV and comparator diseases) from 132 sites in 32 countries around the world.…”