POS1248 DAMAGE IN SJÖGREN’S DISEASE: INSIGHTS FROM THE GIRRCS SJOGREN’S DISEASE COHORT
Authors: A. Marino, O. Berardicurti, I. Genovali, L. Navarini, M. Vomero, D. Currado, L. La Barbera, R. Irace, P. Conigliaro, V. Pavlych, V. Guardo, G. Carlino, R. D. Grembiale, A. Iagnocco, P. Cipriani, M. S. Chimenti, F. Ciccia, G. Guggino, R. Giacomelli
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Primary Sjögren's disease (SD) is a systemic autoimmune disorder characterized by a lymphocytic infiltrate affecting exocrine glands, resulting in gland dysfunction and sicca symptoms. While oral damage is commonly acknowledged, end-organ damage is considered relatively rare in SD patients. Presently, no specific treatment is available for SD, and the diagnostic delay is typically at least 3 years after the onset of symptoms. These characteristics, coupled with the broad spectrum of clinical manifestations, contribute to the complexity of disease management. Despite the generally low rate of disease progression, patients may still encounter end-organ damage and a decline in the quality of life during the course of the disease. In this study, we present data from an Italian cohort designed to evaluate damage accrual over a 5-year period. <h3>Objectives:</h3> This study aims to assess: Systemic, ocular, and oral damage development in a large multicentre cohort of SD patients Risk factors associated with damage presence and development over a 5-year period <h3>Methods:</h3> The GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale) Sjögren's disease cohort is an inception cohort that recruited 448 consecutive SD patients. These patients were enrolled in tertiary rheumatology centers across Italy, each with expertise in SD diagnosis and management. All patients were ≥ 18 years old, and fulfilled the 2016 American–European Consensus Criteria for SS. The Sjogren's syndrome damage index (SDI) was employed to evaluate damage both at baseline and during the follow-up period. Due to missing data, our statistical analysis encompassed 429 patients. Survival analysis was conducted to describe the survival rate and identify risk factors associated with damage development. <h3>Results:</h3> At the enrolment, the median SDI was 0.69 (SD ± 0.93). Over half of the patients exhibited damage at baseline (225 patients, 52.4%). The most common domain of reported damage at baseline was the ocular domain (25.8%), followed by the systemic and oral domains (21.9% and 13.4%, respectively). Higher systemic SDI at baseline was associated with ESSDAI (r = 0.60, p &lt; 0.0001), ESSPRI (r = 0.15, p = 0.0079), and CRP (r = 0.14, p = 0.0011), anti-Ro autoantibodies (r = 0.10, p 0.036), recurrent oral infections (r = 0.17, p = 0.0006). Ocular damage at baseline associated with impaired Schirmer test I (r = 0.20, p &lt; 0.0001), CRP (r = 0.16, p = 0.0017), ESSDAI (r = 0.14, p = 0.003) and anti-La antibodies (r = 0.10, p = 0.044). Oral damage at baseline was associated with ESSDAI (r = 0.26, p &lt; 0.0001), RF (r = 0.20, p &lt; 0.0001), FS (r = 0.18, p = 0.012), CRP (r = 0.18, p 0.0008), recurrent oral infections (r = 0.16, p = 0.0011), ESSPRI (r = 0.15, p = 0.001), anti-Ro antibodies (r = 0.14, p 0.0039), xerostomia and xeropthalmia (r = 0.11, p = 0.028 and 0.022 respectively). After one year, the estimated survival rates for systemic, ocular, and oral damage development were 73%, 69%, and 86%, respectively. A significant increase in the mean of SDI after 1 year (0.86 ± 1.14, p &lt; 0.0001) was observed. ESSDAI values and CCS usage during the first year of follow-up were risk factors for developing systemic damage over 60 months of observation (HR 1.080; 95% CI 1.02, 1.14; p = 0.0041; HR 3.44; 95% CI 1.22, 9.68; p = 0.019, respectively). CCS usage during the first year was a risk factor for ocular damage development (HR 2.16; 95% CI 0.31, 2.48; p = 0.013). Reported data and Kaplan-Meier curves are shown in Figure 1(A–D) and Table 1. <h3>Conclusion:</h3> Our findings suggest that at baseline, more than half of SD patients present systemic, ocular, or oral damage, and damage accumulation persists even after the diagnosis and the initiation of therapy. Different baseline factors, linked to the patients and/or the disease, could contribute to the damage development. The assessment of damage throughout the follow-up of SD patients could prove beneficial in preventing treatment failure and the progression of the disease. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>