Eular recommendations for the management of Sjögren’s syndrome with topical and systemic therapies

Research output: Contribution to journalArticle

Authors

  • Manel Ramos-Casals
  • Pilar Brito-Zeron
  • Stefano Bombardieri
  • Hendrika Bootsma
  • Salvatore De Vita
  • Thomas Dorner
  • Jacques-Eric Gottenberg
  • Gabriela Hernandez-Molina
  • Agnes Kocher
  • Belchin Kostov
  • Aike Kruize
  • Thomas Mandl
  • Wan-Fai Ng
  • Soledad Retamozo
  • Raphaele Seror
  • Yehuda Shoenfeld
  • Antoni Siso-Almirall
  • Athanasios Tzioufas
  • Claudio Vitali
  • Xavier Mariette

Colleges, School and Institutes

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 organizations following the 2014 EULAR standardized operating procedures. The Task Force included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, 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. The Task Force 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 nonsteroidal anti-inflammatory drugs (NSAIDs), topical corticosteroids, topical cyclosporine A, 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 Task Force 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 Task Force. 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

Original languageEnglish
Article number216114
JournalAnnals of the Rheumatic Diseases
Early online date31 Oct 2019
Publication statusE-pub ahead of print - 31 Oct 2019