Search

Sjögren's syndrome is an autoimmune disease characterized by inflammation of the exocrine glands. The disease can be primary or secondary (if it is associated with another autoimmune disease). In Barring symptom management, there is no established treatment. To evaluate the effectiveness and safety of abatacept as a treatment of primary Sjögren's syndrome over the course of 24 months. Eleven patients with primary Sjögren's syndrome from the Rheumatology Department of Universidade Santo Amaro, Sao Paulo, Brazil were enrolled for a prospective observational study. Eligible participants were diagnosed according to the 2002 American-European consensus criteria and had a score greater than 3 on the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). Participants received intravenous abatacept for 24 months at a weight-adjusted dose of 500 mg for patients weighing 60 kg and 750 mg for those weighing 60 kg. The outcomes were ESSDAI activity index, non-stimulated salivary flow rate, ocular dryness (Schirmer test, tear film break-up time, and ocular staining score), SF-36 questionnaire, and Fatigue domain of the FACIT (Functional Assessment of Chronic Illness Therapy) index. There was a statistically significant reduction in ESSDAI index and improvement of salivary flow. One subscale of the SF-36 index-emotional role functioning-showed improvement. There was no change in ocular parameters or in the FACIT index. In this sample of 11 patients with primary Sjögren's syndrome, abatacept therapy improved xerostomia and systemic disease activity.Key Points• Abatacept is safe and effective for the treatment of primary Sjögren's syndrome.• Abatacept can improve salivary flow and ESSDAI index in this patient population.

Background Primary Sjögren’s syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

Background: Primary Sjogren’s syndrome (pSS) is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands and other organs, resulting in dry eye, dry mouth, and systemic findings. [1] Objectives: The American College of Rheumatology/AECG group diagnostic criteria for pSS have included three items related to ocular involvement: The Schirmer test type 1, van Bijsterveld score, and Ocular Staining Score (OSS). They are time-consuming and employ costly dyes and instruments. In this context, we analyzed 66 patients with pSS to ascertain correlation between these tests and assess the possibility of streamlining ophthalmologic examination. Methods: Sixty-six patients (180 eyes) from the Federal University of Sao Paulo outpatient cornea clinic were analyzed prospectively from 2017 to 2018. Those with suspicion of pSS were tested according to the 2016 ACR-EULAR diagnostic criteria. A protocol consisting of the Schirmer, tear-film break-up time (TBUT), van Bijsterveld, and OSS tests was applied to all patients by a cornea specialist. Results were analyzed for correlation and interrater reliability using the kappa coefficient. Results: Of the 66 patients, 64 were female; 36 were white; median age was 53.3 (11,3) years. The Schirmer I test was done in 177 eyes, it was positive in 57.6%. TBUT was tested in 175 eyes and was positive in 96%. The van Bijsterveld score was done in 128 eyes and was positive in 61.7%. The OSS was done in 177 eyes and was positive in 68.9%. All four tests could be performed in 128 eyes. The results were confirmed at the same proportions. Correlation between tests was then calculated through the kappa coefficient (Table 1). Overall agreement was weak (κ = 0.340, p Conclusion: Schirmer’s test without anesthesia has been part of the AECG diagnostic criteria for pSS since 1989. [2]. Our study demonstrates the low sensitivity of the Schirmer I test and the possibility of removing it from the diagnostic criteria. Limitations include our small sample size and lack of comparison group. We hope that, in future, use of objective tests for pSS will become routine ophthalmology practice, reducing costs and facilitating diagnosis. References [1] Shiboski CH, Shiboski SC, Raphaele S, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren’s syndrome. Ann Rheum Dis 2016;0:1–8. [2] Workshop on Diagnostic Criteria for Sjogren’s syndrome. I. Questionnaires for dry eye and dry mouth. II. Manual of methods and procedures. Clin Exp Rheumatol 1989;7:212–19. Disclosure of Interests: None declared

Introduction: Primary Sjogren’s syndrome (SSp) is a systemic autoimmune disease concerning the exocrine glands and internal organs. Therapeutic options mainly include symptomatic and supportive measures, whereas traditional immunosuppressive drugs have not presented efficacy in randomized trials. Abatacept is a T-cell co-stimulation modulator and there are evidences that this drug may be effective for treating Pss. Objectives: The objective of this trial was to evaluate effectiveness and safety of abatacept for 24 months in the treatment of pSS. Methods: Observational prospective study for 24 months. It was included 11 patients that filled the American-European Consensus criteria (2002). Patients received abatacept according the weight Results: This study was constituted from 11 women between the ages 25 to 81 years (average: 53.73 ± 15.07 years), being the majority afro-descendant women (54.5%). Of this total, 90.9% presented positive antinuclear antibody (ANA), 81.8% positive anti/SSA, and 90.9% positive rheumatoid factor. In relation to ESSDAI, 81.8% of the participants presented scores greater than 5. There was a statistically significant reduction in the ESSDAI IC 2.99 [-0.49; 7.99] (p = 0.013). Between the sub items of the ESSDAI, the ones that demonstrated the greatest modifications were articular and glandular. There was a significant increase in the salivary flow of the participants (dif = -0.90; IC-95%: -1.50; -0.50] after 24 months of treatment. There was no statistical difference between the beginning and after 24 months of treatment in the different evaluations, Schimer’s ophthalmologic right eye IC 2.26 [-3.50; 12.5] p=0.423, Break up time OD 0.50 [-1.00; 2.49] p=0.307, OSS right eye IC: 0.50 [-2.50; 5.50] p=0.721. In the SF36 only in the Limitation for Emotional Aspects sub scale reported significant improvement (dif = 36.4; IC95%: -67.1; -5.57; p = 0.025), and FACIT presented no evidences of significant changes between pre and post treatment p = 0.356. Conclusion: The study demonstrates limitations due to the few number of patients included and for being an open study not controlled, but we observed a statistically significant reduction in the ESSDAI, increase in the salivary flow and in the sub scale Limitation for Emotional Aspects of the SF36, indicating a positive effect in the disease. References [1] Clair E.W. ST. Sjogren’s syndrome in: Gary S. Firestein MD, Ralph C. Budd MD, Sherine E. Gabriel MD MSc, et al.Kelley & Firestein’s Textbook of Rheumatology, Tenth Edition. Elsevier 2017 73:1221-44. [2] Tsuboi H, Matsumoto I, Hagiwara S, et al. Effectiveness of abatacept for patients with Sjogren’s syndrome associated with rheumatoid arthritis. An open label, multicenter, one-year, prospective study: ROSE (Rheumatoid Arthritis with Orencia Trial toward Sjogren’s syndrome Endocrinopathy) trial. Mod Rheumatol 2016; 26(6):891-899 Disclosure of Interests: None declared