AB0566 POLYAUTOIMMUNITY AND MAJOR ORGAN INVOLVEMENT PREVALENCE IN SJÖGREN’S SYNDROME: THYROID, LIVER, LUNG AND KIDNEY AS TARGETS. A SINGLE CENTER CROSS SECTIONAL STUDY
Authors: Larissa Valor, Hannah Schenker, Melanie Hagen, Johannes Knitza, Jürgen Rech, Georg Schett
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Polyautoimmunity has been described to be associated with primary Sjögren's syndrome (SjS) and the most frequent observed associated autoimmune diseases (AID) are autoimmune thyroid disease, autoimmune hepatitis and primary biliary cirrhosis, which are common organ-specific AID. In the same track, renal and lung involvement has increasingly been documented in SjS further highlighting its systemic nature. <h3>Objectives</h3> To describe and classify prevalence of polyautoimmunity and major organ involvement in a primary SjS-cohort. <h3>Methods</h3> This cross-sectional study included 179 patients [160 (89%) females and 19 (11%) males] diagnosed with primary SjS and fulfilling the ACR classification criteria (1) that had been admitted to our outpatient clinic between December 2008 and December 2018. Demographic and disease-specific characteristics were recorded in all patients. <h3>Results</h3> In our cohort the median age at diagnosis was 57 years (range: 20-85). Thyroid AID was found in 55/179 (30%) patients, with the following distribution: Hashimoto thyroiditis without (n=21) and with hypothyroidism (n=22), Graves's disease without (n=4) and with thyroidectomy (n=8). Liver AID was detected in 8/179 patients (4%), 3 patients with autoimmune hepatitis and 5 patients with primary biliary cirrhosis. Regarding major organ involvement, 20/179 (11%) patients had renal manifestations: renal insufficiency (n=12), glomerulonephritis (n= 3), interstitial nephritis (n=2) and IgA nephritis (n=3). Eight/179 (4%) patients had lung manifestations: interstitial fibrosis (n=6), emphysema (n=1) and chronic obstructive pulmonary disease (n=1). <h3>Conclusion</h3> Our results add evidence for the presence of polyautoimmnunity and major organ involvement in SjS. We found a slightly lower prevalence of polyautoimmunity and major organ involvement compared to recently reported data (2). Nonetheless, extra-glandular organ involvement should be assessed in order to elucidate cumulative damage and how it might impact outcome, prognosis and therapeutic approaches in SjS. <h3>References</h3> [1] Shiboski SC, et al. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 2012;64(4):475-87. [2] Both T, et al. Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment. Int J Med Sci. (2017) 23;14(3):191-200. <h3>Disclosure of Interests</h3> Larissa Valor: None declared, Hannah Schenker : None declared, Melanie Hagen: None declared, Johannes Knitza: None declared, Jürgen Rech Grant/research support from: Bristol-Myers Squibb and Celgene (greater than $10,000), Consultant for: Bristol-Myers Squibb, Celgene, Chugai, GlaxoSmithKline, Janssen, Eli Lilly, Novartis, Roche, Sanofi Aventis, and UCB (in total more than $10,000), Speakers bureau: Bristol-Myers Squibb, Celgene, Chugai, GlaxoSmithKline, Janssen, Eli Lilly, Novartis, Roche, Sanofi Aventis, and UCB (in total more than $10,000), Georg Schett: None declared