Anti-centromere protein B antibody positivity in primary Sjögren's disease: clinical features and prognostic implications.

To investigate the clinical and immunological features of primary Sjögren's disease (pSjD) patients with anti-centromere protein B (CENP-B) antibody positivity and to evaluate its prognostic significance. This ambispective cohort study included 1,222 patients with pSjD from the China-Japan Friendship Hospital between February 2014 and February 2023, with follow-up through February 2024. Patients were categorized into anti-CENP-B positive and negative groups based on serum testing. Clinical characteristics, immunological features, and outcomes were compared between groups. A subgroup analysis compared patients with isolated CENP-B positivity to those with additional autoantibodies. Statistical analyses were conducted using SPSS 26.0 and R 4.2.3, including descriptive statistics, univariate tests, and Kaplan-Meier survival analysis. In this study, 100 patients (8.2%) were positive for anti-CENP-B antibody, while 1,122 patients (91.8%) were negative. Compared with the negative group, positive patients were older, more often female, and have higher rates of xerostomia and Raynaud's phenomenon, but lower frequency of dyspnea. They showed lower platelet counts, higher aspartate aminotransferase, gamma glutamyl transferase, lactate dehydrogenase, total bilirubin, and lower estimated glomerular filtration rate, with reduced frequencies of elevated Immunoglobulin (Ig) G, but increased IgM levels and slightly lower EULAR Sjögren's Syndrome Disease Activity Index scores. Among anti-CENP-B positive patients, those with isolated anti-CENP-B positivity had milder immunologic abnormalities than those with concomitant autoantibodies. Among patients with available LSGB data, focal lymphocytic infiltration counts were higher in the anti-CENP-B-positive group. Anti-CENP-B positivity was not significantly associated with mortality, cancer, or interstitial lung disease. Anti-CENP-B positivity in pSjD may identify a different baseline serological and clinical profile, characterized by relatively milder immunologic abnormalities and lower disease activity. However, its prognostic and clinical significance remains uncertain and requires further validation.