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

Research output: Contribution to journalArticle

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.

Details

Authors
  • Manuel Ramos-Casals
  • Pilar Brito-Zerón
  • Stefano Bombardieri
  • Hendrika Bootsma
  • Salvatore De Vita
  • Thomas Dörner
  • Benjamin A Fisher
  • Jacques-Eric Gottenberg
  • Gabriela Hernandez-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
  • EULAR-Sjögren Syndrome Task Force Group
Organisations
External organisations
  • University of Pisa
  • University of Groningen
  • University Hospital 'Santa Maria della Misericordia'
  • Charité - University Medicine Berlin
  • University Hospitals Birmingham
  • Bern University Hospital
  • University Medical Center Utrecht
  • Skåne University Hospital
  • National University of Cordoba, Argentina
  • Saint Petersburg State University
  • National and Kapodistrian University of Athens
  • Institutd' Investigacions Biomèdiques August Pi iSunyer (IDIBAPS)
  • Hospital CIMA
  • University Hospital Of Strasbourg
  • University of Strasbourg
  • Salvador Zubirán National Institute of Health Sciences and Nutrition
  • Polytechnic University of Catalonia
  • NIHR Newcastle Biomedical Research Centre
  • Bicêtre Hospital
  • Assistance Publique des Hôpitaux de Paris
  • El Consorci d’Atenció Primària de Salut Barcelona Esquerra
  • Villa San Giuseppe Santo Stefano Riabilitazione, Como
  • Hospital Clínic of Barcelona
  • University of Birmingham
  • University of Bern
  • University of Newcastle upon Tyne
  • Instituto Universitario de Ciencias Biomédicas de Córdoba
  • Sheba Medical Center
  • University of Paris-Sud
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Pages (from-to)3-18
JournalAnnals of the Rheumatic Diseases
Volume79
Early online date2019 Oct 31
Publication statusPublished - 2020
Publication categoryResearch
Peer-reviewedYes

Bibliographic note

© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.