ACR/EULAR classification criteria for eosinophilic granulomatosis with polyangiitis

The ACR/EULAR classification criteria for eosinophilic granulomatosis with polyangiitis is a diagnostic tool designed to identify patients with this specific form of vasculitis. The criteria are intended to provide a standardized approach to diagnosis, facilitating early intervention and appropriate treatment. The criteria incorporate a combination of clinical, laboratory, and imaging findings. The entry criteria include evidence of small- or medium-vessel vasculitis on imaging and the exclusion of alternative diagnoses that mimic vasculitis. These two criteria contribute a significant portion of the total score, reflecting the importance of these findings in the diagnosis of eosinophilic granulomatosis with polyangiitis. The clinical criteria include obstructive airway disease, nasal polyps, and mononeuritis multiplex. These are common manifestations of the disease and their presence contributes to the overall score. Laboratory and biopsy criteria include a blood eosinophil count of 1×10**9/L or higher and extravascular eosinophilic-predominant inflammation on biopsy. These findings are indicative of the eosinophilic inflammation characteristic of the disease. The presence of cytoplasmic antineutrophil cytoplasmic antibodies or anti-proteinase 3 antibodies, as well as hematuria, are considered negative criteria and subtract from the total score, as they are more commonly associated with other forms of vasculitis. The diagnostic criteria are met if the total score is 46 or higher. This threshold was established to ensure a high level of specificity for the diagnosis of eosinophilic granulomatosis with polyangiitis. If the score is below this threshold, the diagnostic criteria are not met, suggesting that an alternative diagnosis should be considered.

Reference

Peter C Grayson, Cristina Ponte, Ravi Suppiah et al. 2022 American College of Rheumatology / European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022 Mar;81(3):309-314.