AB0604 Initial clinical and immunological factors associated with manifestations in patients with primary sjÖgren’s syndrome: a single centre retrospective study
Authors: M. Tsukamoto, K. Suzuki, T. Takeuchi
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests various signs and symptoms. Although a few studies have focused on these manifestations over the long term,<sup>1,2</sup> the association of initial clinical and immunological factors with subsequent longer-term manifestations has not been fully elucidated. <h3>Objectives</h3> To identify initial clinical and immunological factors associated with manifestations in patients with pSS. <h3>Methods</h3> A retrospective review was performed on pSS patients followed over a 10 year period at our department. Clinical and immunological data, including levels of serum immunoglobulin (Ig) and autoantibodies, were collected and statistically analysed. <h3>Results</h3> A total of 224 patients diagnosed with pSS who had met the classification criteria<sup>3</sup> were enrolled. Among them, 201 patients were diagnosed with pSS at our hospital. Of these, we followed the 91 patients who continued to visit our hospital over 10 years. Of the other 110 patients, 69 suddenly interrupted treatment, 20 visited different hospitals, and 13 interrupted treatment at our department and visited dentistry or ophthalmology departments. During observation, 7 patients were newly diagnosed with rheumatoid arthritis in addition to SS and one patient died. We then analysed the 91 patients who continued to visit. Of these, 88 were female and 3 were males. Average age was 52 years. 72 and 33 patients had anti-SS-A antiabody and rheumatoid factor (RF). 82, 68 and 7 patients had neutropenia, anaemia and thrombocyteopenia, respectively. 15% of patients used corticosteroid and/or immunosuppressant treatment. 10% of patients took traditional Chinese medicine. On follow-up for 10 years, titers of IgG, A and M were significantly decreased, whereas complement levels were elevated. The proportion of patients with extraglandular involvement decreased from 90% to 73%, whereas 14% of patients had new extragladular organ involvement. The frequency of extraglandular involvement at 10 years was high in patients with hyper IgG at the initial test (39% vs 85%, p&lt;0.01). The frequency of extraglandular organ involvement at 10 years was high in patients who were RF-positive at diagnosis (3% vs 15%, p&lt;0.05). 9% of patients developed malignancies. 29% of patients without RF at the initial test had RF during the 10 years observation. The presence of dry eyes or mouth findings did not change during follow-up in pSS patients. We then created a multivariate model of predictors for malignancies, extraglandular involvement and extraglandular organ involvement with possible variables at diagnosis. Age, anti-centromere antibody, hyper IgG and anaemia were identified to be significant variables associated with malignancies. Extraglandular involvement was associated with the presence of hyper IgG (p&lt;0.01), and extraglandular organ involvement was associated with RF positivity (p&lt;0.05). <h3>Conclusions</h3> Our study newly identified initial clinical and immunological factors associated with manifestations in patients with pSS over a long period. pSS patients with RF and hyper IgG at diagnosis were candidates for the development of extraglandular involvements in the future. <h3>References</h3> [1] Clin Med (Lond) 2014; 14: 157–64, [2] Clin Exp Rheumatol 2004; 22: 55, [3]Ann Rheum Dis2017; 76: 9–16. <h3>Disclosure of Interest</h3> None declared