SAT0215 HISTORY OF TONSILLECTOMY IS ASSOCIATED WITH GLANDULAR INFLAMMATION IN SJÖGREN’S SYNDROME

Abstract

Background: The palatine tonsils are secondary lymphoid-organs that serve as the first line of defense against pathogens. Whether history of tonsillectomy (TE) is associated with the phenotype of Sjögren's syndrome (SjS) has not been investigated to date. Objectives: To test whether TE is linked to SjS phenotype and disease activity scores. Methods: A total of 183 patients from the Optimising Assessment in Sjögren's Syndrome (OASIS) cohort with SjS or non-SjS sicca syndrome were analysed. Patients with SjS fulfilled 2016 ACR/EULAR classification for primary SjS; sicca patients had objective and/or subjective dryness, but were anti-Ro/SSA negative and had no physician diagnosis of SjS. One SjS patient who had TE around the time of symptom onset was excluded. Results: Of the total cohort, 116 were diagnosed with SjS (86.2% SSA/Ro positive) and 67 with non-SjS sicca syndrome. Overall, 29% (53/183) had TE; 24.1% of the SjS patients (28/116) and 37.3% of the sicca patients (25/67). The prevalence of TE was higher in sicca than in SjS (p=0.043). The median age at TE was 8 (range 3-50) years and did not differ between SjS and sicca patients (p=0.629). Neither age at first symptoms (p=0.093) nor disease duration (p=0.623) were associated with TE in patients with SjS. SjS patients with TE showed a higher average histological focus score (2.1 (1.2-2.8) vs. 1.3 (0.0-4.3); p=0.049), and were more likely to have activity in the glandular (53.6 vs. 20.5%; p=0.001) and constitutional (39.3 vs. 14.9%, p=0.014) domains of the ESSDAI, and lower levels of IgG (12.2 (7.8-35.6) vs. 15.6 (5.7-56.4) g/l; p=0.012) and IgA (2.3 (0.9-6.6) vs. 2.9 (0.7-9.4) g/l; p=0.032). Whereas there was no difference in EQ5D utility values (p=0.718), VAS global health was significantly lower in the patients with SjS who had TE (58 (10-78) vs. 70 (10-97); p=0.021). There was no association between the status of TE and autoantibodies (SSA, SSB, RF), lachrymal and salivary glands function (Schirmer's test, unstimulated saliva flow), complement (C3, C4), serum levels of free light chains, β2-microglobulin, ESSPRI, or total ESSDAI (all p-values >0.1). Of 181 patients, 12.7% (23/283) had appendectomy (AE); 10.5% (12/114) of the SjS patients and 16.4 %(11/67) of the sicca patients (p=0.258). With the exception of lower unstimulated salivary flow (0.086 (0.01-0.43) vs. 0.11 (0.0-1.3) ml/min; p=0.026) in SjS patients with AE, there were no differences in disease phenotype between SjS patients with and without AE (all p-values >0.1). Conclusion: History of TE in SjS is associated with higher average focus scores and with glandular swelling. It could be speculated that the absence of palatine tonsils is compensated by enhanced lymphocytic infiltrates in the salivary glands. Further research is required to determine if TE is a risk factor for both SjS and non-SjS sicca and to determine the role of the tonsils in the generation of hypergammaglobulinaemia in SjS.