AB0487 SJÖGREN SYNDROME: DESCRIPTIVE AND COMPARATIVE STUDY OF PRIMITIVE AND ASSOCIATED FORMS

• Published in: Annals of the rheumatic diseases Vol. 81; no. Suppl 1; pp. 1370 - 1371
• Main Authors: Skhiri, S., Baya Chatti, A., Ben Yahia, W., Atig, A., Ghannouchi, N.
• Format: Journal Article
• Language: English
• Published: 01.06.2022
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2022-eular.1853

Background Sjögren syndrome (SS) is an autoimmune disease (AID) characterized by a dysfunction of the exocrine glands (mainly salivary and tear glands). It is responsible for xerostomia and xerophtalmia as well as systemic manifestations that may affect multiple organs. This syndrome comes in two forms: primary when it is isolated (pSS) and associated (aSS) when it is in association with another AID whether it is specific of organs or not. These two entities have different particularities, requiring explorations and adapted treatments. Few studies have taken interest in determining them. Objectives The objective of this study was to compare the various clinical and evolutionary manifestations of these two forms. Methods We conducted a retrospective analytical study over a period of 33 years. All patients with SS based on ACR 2012 and/or ACR/EULAR classification criteria were included. Epidemiological, clinical, para-clinical, therapeutic and disease course characteristics in both groups (pSS and aSS) were described and compared. Results One hundred and forty-five patients were included. Sixty-three (43.4%) had pSS and 82 (56.5%) had aSS. The pSS group consisted of 58 women (92%) and five men (8%) with a mean age at diagnosis of 51.9 ±15.3 years. Seventy-eight women (95%) and four men (5%) with a mean age at diagnosis of 45.4±16.2 years had a SS. The most common AADs associated with the latter were SLE (39%) and RA (18.3%). The comparison of the two groups concluded that the mean age at the time of diagnosis was significantly higher in patients with pSS (p= 0.01). Glandular involvement was almost constant in both groups. Joint involvement, skin involvement and Raynaud’s syndrome were significantly more common in the aSS group (p< 0.01; < 0.001 and 0.02 respectively). Central neurological involvement was significantly more common in the pSS group (p=0.04). The other clinical manifestations were comparable in both groups. Comparison of biological abnormalities between pSS and aSS showed a statistically higher frequency of leukopenia (p=0.04), lymphopenia (p <0.001), Antinuclear antibodies (p=0.001), anti SSA antibodies (p=0.003), rheumatoid factor (p < 0.001) and anti CCP antibodies (p<0.001) in aSS. Synthetic antimalarial drugs, corticosteroids and immunosuppressive treatment were significantly more prescribed for aSS (p= 0.003, p < 0.001 and p=0.02 respectively). After a mean of follow up of 3 ±4.5 years for the pSS and 4 ±4.5 years for the aSS. No cases of lymphoma were observed in both groups. Conclusion Differentiating between pSS and aSS is crucial in order to establish an early diagnosis and ensure adequate management and a better prognosis. Larger-scale studies appear to be needed to optimize management. Disclosure of Interests None declared