AB0834 ANTICENTROMERE ANTIBODIES IN THE SETTING OF SJÖGREN SYNDROME: ANALYSIS OF A MULTICENTRIC COHORT
Authors: M. R. Pellico, C. Iannone, C. Baldini, R. Priori, I. Fischetti, N. D ’angelo, F. Cacciapaglia, S. Stano, P. Cipriani, V. Pavlych, F. Carubbi, A. Alunno, M. T. Rizzo, L. Quartuccio, O. Berardicurti, F. Atzeni, S. Cavalli, A. Minniti, E. Bartoloni, R. F. Caporali, N. Del Papa
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> In addition to anti-SSA and anti-SSB antibodies, considered hallmarks of Sjogren Syndrome, recent studies described several other autoantibodies in the disease, but the clinical associations are not yet well known. Particularly, anti-centromere antibodies (ACA) have been increasingly studied in the context of primary Sjögren Syndrome (pSS). Approximately 3-5% of pSS patients show positivity for ACA. Different studies from national registries or data collections examined the clinical phenotype of ACA-positive SS patients with contrasting results, then definitive characterizations of this subgroup remain to be established <h3>Objectives:</h3> To describe the clinical characteristics of an Italian multicenter cohort of ACA positive (ACA+) pSS patients and compare them to a matched ACA-negative (ACA-) pSS cohort and an ACA+ Systemic Sclerosis (SSc) cohort. <h3>Methods:</h3> A retrospective analysis was conducted on patients meeting the ACR/EULAR SS classification criteria showing ACA positivity, referring to participating centers. Organ involvement and related clinical, instrumental and laboratory characteristics were evaluated. <h3>Results:</h3> The cohort comprised 55 ACA+ patients diagnosed with pSS, including 36 females, with a mean age of 69.15 years. Predominant clinical manifestations were Raynaud's phenomenon (n=45), arthralgias (n=37), xerostomia (n=48), and xerophthalmia (n=39). Comparison to an ACA- pSS cohort revealed no significant demographic variance. However, the ACA+ cohort exhibited significantly higher incidence of Raynaud's phenomenon, puffy fingers, scleroderma, telangiectasia, and gastrointestinal involvement (p&lt;0.001, 0.0003, 0.0025, &lt;0.0001, and 0.01, respectively) as shown in Table 1. In contrast to literature, no significant anti-Ro/SSA antibody prevalence or cancer development differences were observed between ACA+ and ACA- groups. We further compared the ACA+ pSS cohort to 96 ACA+ SSc patients. The latter demonstrated higher frequency of manifestations like scleroderma, puffy fingers, Raynaud's, telangiectasias, calcinosis, pitting scars, and acral ulcers (p&lt;0.0001, &lt;0.0001, &lt;0.0001, 0.003, 0.008, &lt;0.0001, &lt;0.0001, respectively). Conversely, the ACA+ pSS cohort exhibited more fibromyalgia, xerostomia, xerophthalmia, and anti-Ro/SSA positivity (p=0.002, &lt;0.0001, &lt;0.0001, &lt;0.0001, respectively), as shown in Table 2. <h3>Conclusion:</h3> Our study shows that in a large cohort of Italian pSS patients, ACA positivity significantly influences disease clinical expression, with a more pronounced pSS-related extraglandular phenotype compared to ACA- pSS patients. However, when compared to SSc patients, ACA+ pSS patients display a less severe vascular profile, resulting in an intermediate clinical phenotype between limited cutaneous scleroderma and pSS. ACA presence in pSS patients may play a pivotal role in clinical features across disease settings. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>