Background Primary Sjögren's syndrome (pSS) is a multisystem autoimmune disease that encompasses extraglandular features that are traditionally managed with steroids and immunosuppresors.
Objectives To assess the use of prednisone (PDN) and immunosuppressors in a retrospective pSS cohort and to evaluate their association with damage accrual.
Methods We reviewed the medical records of 155 pSS patients who regularly attended our Institution (2010-2014) with at least 6 months of follow-up. We used a standardized form to register demographics, glandular (oral/ocular symptoms, parotid enlargement) and extraglandular features. We specifically reviewed data regarding the use of PDN and immunosuppresors from the diagnosis until the last visit. We also scored the SSDI at the last visit (n=116). We used descriptive statistics, Chi-square test and logistic regression analysis reporting OR and 95%C.I.
Results Patients were 92.2% females, mean age 57.4±14.7 years and median follow-up 11 years (0.5-47.9). Fifty-one patients (32.9%) were free of treatment and 104 (67%) had ever received PDN and/or immunosuppressors. The latter group presented more parotid enlargement (55.8% vs. 37.3%, p=0.04), lymphadenopathy (34.6% vs. 13.7%, p=0.007), arthritis (45.2% vs. 9.8%, p<0.0001), autoimmune hepatitis (9.6% vs. 0%, p=0.02), pulmonary (16.3% vs. 3.9%, p=0.03) and hematologic involvement (44.2% vs. 25.5%, p=0.03). The percentage of use, median number of use periods, maximum dose, main indication and percentage of toxicity are shown at Table 1. Some patients with isolated parotid enlargement (PDN 3%, AZA 2%, antimalarial 1%, MTX 0.6%) or oral/ocular dryness (PDN 1.9%, antimalarial 7%, AZA and MTX 0.6% for each) also received treatment. The overall median SSDI score was 3 (0-8). Patients with a SSDI>2 (n=60) used more frequently cyclophosphamide (15% vs. 0%, p=0.003) and less often antimalarials (35% vs. 55%, p=0.02). At the regression logistic analysis adjusted by disease duration, a SSDI >2 was associated with the presence of any extraglandular feature (OR 4.4, 95% C.I. 1.39-13.9, p=0.01) whereas the use of antimalarials was protective (OR 0.36, 95% C.I. 0.16-0.82, p=0.01).
Conclusions More than 50% of pSS patients received PDN and/or immunosuppresors during their follow-up. This fact confirms the systemic nature of the disease and should alert physicians of secondary adverse events with the use of this treatment. The use of antimalarial seemed to protect against damage accrual, an observation that needs further validation in another cohorts.
Disclosure of Interest None declared