2019 EULAR RECOMMENDATIONS FOR THE MANAGEMENT OF SJöGREN”S SYNDROME WITH TOPICAL AND SYSTEMIC THERAPIES

Manuel Ramos-Casals, Pilar Brito-Zerón, Stefano Bombardieri, Hendrika Bootsma, Salvatore De Vita, Thomas Dörner, Benjamin A. Fisher, Jacques-Eric Gottenberg, Gabriela Hernández-Molina, Agnes Kocher, Belchin Kostov, Aike A. Kruize, Thomas Mandl, Wan-Fai Ng, Soledad Retamozo, Raphaèle Seror, Yehuda Shoenfeld, Antoni Sisó-Almirall, Athanasios G. Tzioufas, Claudio Vitali, Simon Bowman, Xavier Mariette on behalf of the EULAR-Sjögren Syndrome Task Force GroupDepartment of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Barcelona, Spain University of Barcelona, Hospital Clínic, Barcelona, Spain Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain Rheumatology Unit, University of Pisa, Pisa, Italy Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital “Santa Maria della Misericordia”, Udine, Italy Karl Landsteiner Institute for Physical Medicine and Rehabilitation, and Department of Physical Medicine and Rehabilitation, Kaiser Franz Josef-Hospital, SMZ Süd, Vienna, Austria Rheumatology Department, University Hospitals Birmingham NHS Foundation Trust, National Institute for Health Research, Birmingham Biomedical Research Centre, Birmingham, United Kingdom Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico Department of Rheumatology, Immunology and Allergology, Inselspital University Hospital Bern, Switzerland and Institute of Nursing Science (INS), Department Public Health (DPH), Faculty of Medicine, University of Basel, Switzerland Primary Healthcare Transversal Research Group, IDIBAPS Barcelona, Spain Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, United Kingdom Instituto Modelo de Cardiología Privado S.R.L., Córdoba, Argentina, IUCBC, INICSA, CONICET, Córdoba, Argentina Université Paris-Sud; Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Department of Rheumatology, INSERM U1184, Le Kremlin Bicêtre, France Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Aviv University, 5265601, Tel Hashomer, Israel Centre d”Assistència Primària ABS Les Corts, CAPSBE, Barcelona, Spain Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece Villa San Giuseppe, Istituto S. Stefano, Como, ItalyAims: The therapeutic management of Sjögren syndrome (SjS) has not changed substantially over the past decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed to develop the first EULAR evidence-, consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organizations following the 2014 EULAR standardized operating procedures. Methods: The Task Force included rheumatologists, specialists in internal medicine, oral care specialists, ophthalmologists, gynaecologists, dermatologists, epidemiologists, statisticians, GPs, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. On the basis of the research questions, a systematic literature search between January 1986 and December 2017 was carried out. Summary-of-findings tables were generated and levels of evidence were determined according to the study design using the standards of the Oxford CEBM. A web-based Delphi procedure was carried out in order to reach consensus. For each statement, the grade of recommendation was based on the maximum level of evidence achieved in the SLR using the 4-point scale CEBM categorization. Consensus for endorsement was defined as an agreement score of at least 8 on a 0-10 scale by more than 80% of participants. Results: The Task Force endorsed the presentation of general principles for the management of patients with SjS as 3 overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic, extraglandular disease ( Table 1 ). Conclusion: The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the area of management of primary 2002 SjS patients and on discussions by a large and broadly international Task Force. The recommendations synthesise the current thinking on approaching SjS treatment in a set of overarching principles and recommendations. We hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations. Abstract SP0190 – Table 1 Overarching and specific recommendations. LoA (% endorsement) LoE GoR A. Patients with SjS should be managed at, or in close collaboration with, centres of expertise following a multidisciplinary approach 90,7 na Na B. The first therapeutic approach for dryness should be symptomatic relief using topical therapies 93,3 na Na C. Systemic therapies may be considered for treating active systemic disease 90,7 na Na 1. Baseline evaluation of salivary gland function is recommended before starting treatment for oral dryness 81,3 5 D 2. The preferred first therapeutic approach for oral dryness according to salivary gland function may be: 2.1. Non-pharmacological stimulation for mild dysfunction; 2.2. Pharmacological stimulation* for moderate dysfunction; 2.3. Saliva substitution for severe dysfunction 88,0 1a/*1b B 3. First-line therapeutic approach of ocular dryness includes the use of artificial tears and ocular gels/ointments 98,7 1a B 4. Refractory/severe ocular dryness may be managed using topical immunosuppressive-containing drops* and autologous serum eye drops 94,7 1a/*1b B/D 5. Concomitant diseases should be evaluated in patients presenting with fatigue/pain, whose severity should be scored using specific tools 93,3 5 D 6. Consider analgesics or other pain-modifying agents for musculoskeletal pain considering the balance between potential benefits and side-effects 89,3 4 C 7. Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions 89,3 4 C 8. Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease 85,3 4 C 9. Immunosuppressive agents should be mainly used as GC-sparing agents, with no evidence supporting the choice of one agent over the others 82,7 4 C 10. B-cell targeted therapies may be considered in patients with severe, refractory systemic disease 98,7 1b D 11. Systemic organ-specific therapeutic approach may follow as a general rule the sequential (or combined) use of glucocorticoids, immunosuppressive agents and biologics 98,7 5 D 12. Treatment of B-cell lymphoma should be individualized according to the specific histological subtype and disease stage 88,0 4 C Disclosure of Interests: None declared DOI: 10.1136/annrheumdis-2019-eular.8570Citation: Ann Rheum Dis, volume 78, supplement 2, year 2019, page A59Session: EULAR Projects in clinical affairs (Speakers Abstracts)