AB0674 PRIMARY SJOGREN SYNDROME WITH ANTICENTROMERE ANTIBODIES - A CLINICALLY DISTINCT PHENOTYPE OR AN OVERLAP SYNDROME?

Background Anticentromere antibodies (ACA) have been reported in primary Sjogren syndrome (pSS) at lower frequencies but recently suggested as another clinically important marker this disease 1 . Although several papers published on ACA-positive pSS, the results conflicting 2,3 Objectives To evaluate correlation between presence of ACA and associated symptomatology, especially related to microvascular dysfunction a Romanian cohort patients with pSS. Methods We assessed 132 who met 2016 American College Rheumatology/European League Against Rheumatism classification criteria for For all ACA+ patients, we collected data sclerodactyly, puffy hands, Raynaud’s phenomenon, calcinosis, telangiectasia, esophageal involvement, interstitial lung disease, digital ulcerations/pitting scars, pulmonary hypertension nailfold capillaroscopy findings. Results The prevalence our pSS was 13%. - positive were older than ACA-negative (median, 59 years [IQR, 48 62] vs median, 50 45 60]; p = 0.042), shorter duration (53 months 260 months, p=0.073). Most female (98.7%) had ocular oral symptoms dryness (98.1% 97.8%, respectively). Among 5 limited cutaneous systemic sclerosis (lcSSc). there no statistical differences two groups, characterized by higher symptomatology dysfunction: RP (Raynaud’s phenomenon) telangiectasias present 50% (5/10), sclerodactyly ulcerations (DUs) 33% (3/10); gangrene not found. 60% (6/10) abnormal findings capillaroscopy, which nonspecific 2 (crossed capillaries), while remaining patient an early-pattern, active-pattern late scleroderma-pattern. positively correlated (p 0.024), DUs 0.034). In study, showed similar severities extraglandular manifestations compared group. Nonetheless, noticed that even if statistically significant, use vasodilators more common (66.7% 12.2%, 0.911) thus supporting hypothesis symptomatic dysfunction. A proportion (64% 42%) treated immunosuppressive agents. No mortality found groups. Conclusion Patients group older, frequent ulcers. cohort, positivity little impact severity patients. significant differences, immunosuppressants agents common. This study is first investigate vascular References [1]Youngjae Park Clinical influences anticentromere antibody Sjögren’s prospective Korean J Intern Med 2021;36(6):1492-1503. [2]Nakamura H, Kawakami A, Hayashi T, et al. Anti-centromere antibody-seropositive Sjogren’s differs from conventional subgroup clinical pathological study. BMC Musculoskelet Disord 2010;11:140. [3]Baer AN, Medrano L, McAdams-DeMarco M, Gniadek TJ. Association severe exocrine glandular syndrome: analysis International Collaborative Alliance Cohort. Arthritis Care Res (Hoboken) 2016;68:1554-1559. Disclosure Interests None declared