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Ann Rheum Dis. 2019 Oct 31. pii: annrheumdis-2019-216114. doi: 10.1136/annrheumdis-2019-216114. [Epub ahead of print]

EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.

Author information

1
Department of Autoimmune Diseases, Hospital Clinic de Barcelona Institut Clinic de Medicina i Dermatologia, Barcelona, Catalunya, Spain mramos@clinic.cat.
2
CELLEX, Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Barcelona, Catalunya, Spain.
3
Autoimmune Diseases Unit, Department of Internal Medicine, Hospital CIMA Sanitas, Barcelona, Catalunya, Spain.
4
Rheumatology Unit, University of Pisa, Pisa, Toscana, Italy.
5
Department of Rheumatology & Clinical Immunology, University of Groningen, Groningen, Groningen, The Netherlands.
6
Clinic of Rheumatology, University Hospital Santa Maria della Misericordia, Udine, Italy.
7
Department of Medicine/Rheumatology and Clinical Immunology and DRFZ, Charite University Hospital Berlin, Berlin, Germany.
8
Institute of Inflammation and Aging, University of Birmingham, Birmingham, UK.
9
National Institute of Health Research Birmingham Biomedical Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
10
Department of Rheumatology, Strasbourg University Hospital, National Reference Center for Rare Systemic Autoimmune Diseases, CNRS, IBMC, UPR 3572, Université de Strasbourg, Strasbourg, Alsace, France.
11
Immunology and Rheumatology Department, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Tlalpan, Mexico.
12
Institute of Nursing Science, Department of Public Health, Faculty of Medicine, University of Basel, Basel, Switzerland.
13
Department of Rheumatology, Immunology and Allergology, Inselspital, University Hospital of Bern, Bern, Switzerland.
14
Primary Healthcare Transversal Research Group, IDIBAPS, Barcelona, Catalunya, Spain.
15
Department of Statistics and Operations Research, Universitat Politecnica de Catalunya, Barcelona, Catalunya, Spain.
16
Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
17
Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden.
18
Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, Tyne and Wear, UK.
19
NIHR Newcastle Biomedical Research Centre, Newcastle upon Tyne NHS Foundation Trust, Newcastle Upon Tyne, Tyne and Wear, UK.
20
Rheumatology Unit. Instituto Modelo de Cardiología Privado SRL, Instituto Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Córdoba, Argentina.
21
Instituto de Investigaciones en Ciencias de la Salud, Consejo Nacional de Investigaciones Científicas y Técnicas (INICSA-UNC-CONICET), Universidad Nacional de Córdoba, Cordoba, Córdoba, Argentina.
22
Center for Immunology of Viral Infections and Autoimmune Diseases, INSERM UMR 1184, Université Paris Sud, Université Paris-Saclay, Paris, France.
23
Department of Rheumatology, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Paris, France.
24
Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Israel.
25
Head of The Mosaic of Autoimmunity Project, Saint Petersburg University, Saint Petersburg, Russian Federation.
26
Primary Care Center Les Corts, CAPSBE, Barcelona, Catalunya, Spain.
27
Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
28
Villa San Giuseppe, Istituto S. Stefano, Como, Italy.
29
Rheumatology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.

Abstract

The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent 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 at developing the first EULAR evidence and 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, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, 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. The TF endorsed the presentation of general principles for the management of patients with SjS as three 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 disease. The recommendations address the use of topical oral (saliva substitutes) and ocular (artificial tear drops, topical non-steroidal anti-inflammatory drugs, topical corticosteroids, topical CyA, serum tear drops) therapies, oral muscarinic agonists (pilocarpine, cevimeline), hydroxychloroquine, oral glucocorticoids, synthetic immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide and mycophenolate), and biological therapies (rituximab, abatacept and belimumab). For each recommendation, levels of evidence (mostly modest) and TF agreement (mostly very high) are provided. The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the management of primary 2002 SjS patients and on discussions between a large and broadly international TF. The recommendations synthesise current thinking on 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.

KEYWORDS:

autoimmune diseases; sjøgren's syndrome; treatment

Conflict of interest statement

Competing interests: MR-C reported consultancy for BMS, Gilead; FB reported consultancy GSK, UCB, ONO, Roche; MB consultancy and/or unrestricted grants from Medimmune, Amgen, GSK, Janssen, AbbVie; SB Participation in Abbvie Advisory Board; RP for Abbvie, Novartis, Ab2 Bio ltd, Celltrion healthcare; RC: speaker’s and/or consultation fee from: Abbvie, BMS, Celgene, Gilead, Janssen Cilag, Lilly, MSD, Novartis, Pfizer, Roche and Sanofi; MR reported consultancy for Abbvie, BMS, Celgene, Janssen Cilag, Novartis, Pfizer, Roche and Sanofi; W-FN reported consultancy for GSK, Novartis, BMS, MedImmune and Abbvie; AGT reported Research Grants From Pfizer, Novartis, Abbvie, Genesis, GSK, Janssen, Eli-Lilly, Through The Research Accounts Of The University Of Athens; CHS: Consultant for Novartis in 2018; PW consultant for Roche, Novartis, Pfizer, Abbvie, Lilly, Gedeon-Richter, Sandoz, Medac, MSD, Sanofi-Aventis. TM reported consultancy Novartis; BAF reported consultancy for Novartis, Roche, MedImmune, BMS.

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