Ab0584 not all sjögren is dry – preserved glandular function in a subgroup of patients with primary sjögren‘s syndrome

Angelika Lackner,Sabine Zenz, J. Hermann, J. Thiel, M. Stradner

Annals of the Rheumatic Diseases(2023)

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摘要
Background Lymphocytic infiltration of the salivary and lachrymal glands is a hallmark of primary Sjögren’s Syndrome (PSS). Chronic inflammation leads to progressive destruction of the glands resulting in dryness of the eyes and mouth termed sicca. The course and pace of sicca development in PSS is elusive. Objectives To explore the effect of symptom duration on sicca and disease activity. Methods Data of patients from the PSS registry of the Medical University of Graz fulfilling the 2016 ACR/EULAR classification criteria for PSS were analysed. We analysed disease duration and total duration of sicca symptoms, Schirmer’s test, unstimulated salivary flow test (USF), stimulated salivary flow test (SSF) and ESSDAI (EULAR Sjögren’s Syndrome disease activity index), Evaluators Global Assessment (EGA), and focus score of the initial lip salivary gland biopsy. In addition, salivary gland ultrasound (SGUS) was performed using the De Vita scoring system. Free light chains (FLC) ĸ and λ , IgG, rheumatoid factor (RF) IgA were determined. The patient’s perspective was analysed by ESSPRI and PSS-QoL. Results Data from 159 PSS patients were analysed, of whom 92.6% (n=148) were female. The mean age was 59.3±12.2 years (± standard deviation), with a mean disease duration of 5.5±5.4 years and a mean sicca duration of 11.6±7.4 years. Interestingly, results of USF, SSF, and Schirmer’s Test did not decrease with duration of disease or sicca. The lack of correlation was probably caused by a subset of patients in whom glandular function remained stable 10 to 33 years after the onset of sicca. We defined this subgroup of patients by an SSF >0.5ml/min and with a symptom duration >10 years (SFSD10). 19.5% (n=31) of our PSS patient were categorised into this group. SFSD10 showed a significantly higher Schirmers test (median 0 (0-35] vs 3[3-35], p<0.01), lower ESSPRI-dryness (7 [1-10] vs. 5[1-10], p<0.05), PSS-QoL dryness (11[2-22] vs. 8 [3-15], p<0.01), lower IgG (15.5 [7.4-38.1] vs. 11.2 [6.5-30.5], p<0.05) and FLC-K (25 [3.9-134] vs. 20.2 [10.3-63.6], p<0.05) compared to non-SFSD10-group. SGUS De Vita score was significantly lower in the SFSD10 subgroup (median 1 [0-2] vs. 2 [1-3], p=0.045). We found no difference in ESSDAI, focus score, antibody status, pain, fatigue or HRQL between the two groups. Conclusion Our results indicate the presence of a subgroup of PSS patients with preserved glandular function despite long-standing disease. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests None Declared.
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关键词
sjögren,syndrome,glandular function
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