Ab0861 Reclassification of Overlap Syndrome of Primary Sjögren Syndrome With Anca-Associated Vasculitis According to the New 2022 Classification Criteria for Vasculitis

Background: Systemic vasculitis is one of the most frequent extra-glandular manifestations in patients with Primary Sjögren syndrome (pSS), and further, small vessel vasculitis, particularly cryoglobulinaemic vasculitis is known as the most common vasculitis in patients with pSS Objectives: This study applied the 2022 American College of Rheumatology/European Alliance of Association for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with pSS accompanied by signs and symptoms suggestive of small- and medium-vessel vasculitis and investigated the overall frequency of and the major contributing factors to the reclassification of overlap syndrome of pSS with AAV (OS-pSS-AAV). Methods: This study included 116 patients with pSS according to the inclusion criteria, and defined signs and symptoms suggestive of small- or medium-vessel vasculitides as lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis. The classification could be made when the total scores for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are 5 points or more, and that for eosinophilic GPA (EGPA) is 6 points or more. Results: The median age of the patients was 56.0 years, and 87.1% were women. Ninety-five, 12, and 37 patients had lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis. According to the 2022 ACR/EULAR criteria for AAV, 35 of 116 (30.2%) patients were reclassified as having OS-pSS-AAV. Among these 35 patients, four were reclassified as having both OS-pSS-MPA and OS-pSS- GPA, whereas, one as having both OS-pSS-MPA and OS-pSS-EGPA simultaneously. The major contributing factor to the reclassification of OS-pSS-AAV was ANCA positivity. Conclusion: The overall frequency of the reclassification of OS-pSS-AAV was 30.2% in pSS patients accompanied by signs and symptoms suggestive of small- and medium-vessel vasculitis. Its likelihood increased according to ANCA positivity. REFERENCES: [1] Argyropoulou OD, Tzioufas AG. Common and rare forms of vasculitis associated with Sjögren's syndrome. Curr Opin Rheumatol 2020;32:21-8. [2] Scofield RH. Vasculitis in Sjögren's Syndrome. Curr Rheumatol Rep 2011;13:482-8. Acknowledgements: NIL. Disclosure of Interests: None declared.