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Sjögren syndrome
Review
Efficacy and safety of topical and systemic medications: a systematic literature review informing the EULAR recommendations for the management of Sjögren’s syndrome
  1. Pilar Brito-Zerón1,2,
  2. Soledad Retamozo3,4,
  3. Belchin Kostov5,6,
  4. Chiara Baldini7,
  5. Hendrika Bootsma8,
  6. Salvatore De Vita9,
  7. Thomas Dörner10,
  8. Jacques-Eric Gottenberg11,
  9. Aike A. Kruize12,
  10. Thomas Mandl13,
  11. Wan-Fai Ng14,
  12. Raphaele Seror15,16,
  13. Athanasios G. Tzioufas17,
  14. Claudio Vitali18,
  15. Simon Bowman19,
  16. Xavier Mariette15,16 and
  17. Manuel Ramos-Casals2,20
  1. 1Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain
  2. 2Laboratory of Autoimmune Diseases Josep Font, CELLEX, IDIBAPS, Barcelona, Spain
  3. 3Department of Rheumatology, Instituto Modelo de Cariología Privado S.R.L, Instituto Universitario de Ciencias Biomédicas de Córdoba, Cordoba, Argentina
  4. 4Instituto De Investigaciones En Ciencias De La Salud (INICSA), Universidad Nacional de Córdoba (UNC), Cordoba, Argentina
  5. 5Research Primary Healthcare Transversal Research Group, CAP Les Corts, CAPSBE, IDIBAPS, Barcelona, Spain
  6. 6Statistics and Operations Research Department, Universitat Politecnica de Catalunya, Barcelona, Spain
  7. 7Rheumatology Unit, Universita degli Studi di Pisa, Pisa, Italy
  8. 8Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  9. 9Clinic of Rheumatology, University Hospital Santa Maria della Misericordia, Udine, Italy
  10. 10Department of Medicine/Rheumatology and Clinical Immunology and DRFZ, Charité Universitätsmedizin Berlin Campus Charite Mitte, Berlin, Germany
  11. 11Department of Rheumatology, Strasbourg University Hospital, National Reference Center for Rare Systemic Autoimmune Diseases, CNRS, IBMC, UPR 3572, Université de Strasbourg, Strasbourg, France
  12. 12Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands
  13. 13Department of Rheumatology, Skane University Hospital Malmö, Lund University, Lund, Sweden
  14. 14NIHR Newcastle Biomedical Research Centre, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, UK
  15. 15Department of Rheumatology, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Le Kremlin-Bicetre, France
  16. 16Center for Immunology of Viral Infections and Autoimmune Diseases, INSERM UMR 1184, Université Paris-Sud, Université Paris-Saclay, Paris, France
  17. 17Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
  18. 18Villa San Giuseppe, Istituto S. Stefano, Como, Italy
  19. 19Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  20. 20Department of Autoimmune Diseases, ICMiD, Hospital Clinic de Barcelona, Barcelona, Spain
  1. Correspondence to Dr. Manuel Ramos-Casals; mramos{at}clinic.cat

Abstract

Objective To evaluate current evidence on the efficacy and safety of topical and systemic medications in patients with primary Sjögren syndrome (SjS) to inform European League Against Rheumatism treatment recommendations.

Methods The MEDLINE, EMBASE and Cochrane databases were searched for case-control/prospective cohort studies, randomised controlled trials (RCTs) and systematic reviews.

Results Current evidence in primary SjS patients fulfilling the 2002 criteria is based on the data from 9 RCTs, 18 prospective cohort studies and 5 case-control studies. Two Cochrane systematic literature reviews (SLRs) have reported that topical treatments for dry mouth and dry eye are safe and effective. Ocular cyclosporine A was safe and effective in two RCTs including 1039 patients with dry eye syndrome. Two Cochrane SLRs on serum tear drops and plugs showed inconsistency in possible benefits, both for symptoms and objective measures. Five RCTs reported significant improvements in oral dryness and salivary flow rates for pilocarpine and cevimeline. An RCT showed no significant placebo-differences for hydroxychloroquine 400 mg/day for the primary outcome (visual analogue scale (VAS) composite of dryness, fatigue and pain). We identified seven RCTs carried out in primary SjS patients. RCTs using infliximab, anakinra and baminercept found no placebo-differences for the primary outcomes. The two largest RCTs randomised 255 patients to receive rituximab or placebo and reported no significant results in the primary outcome (VAS composite), while prospective studies suggested efficacy in systemic disease.

Conclusion The current evidence supporting the use of the main topical therapeutic options of primary SjS is solid, while limited data from RCTs are available to guide systemic therapies.

  • Sjøgren's syndrome
  • treatment
  • multidisciplinary team-care

This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See: https://creativecommons.org/licenses/by/4.0/.

https://doi.org/10.1136/rmdopen-2019-001064

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    Footnotes

    • Twitter @@ramos_casals

    • Contributors PB-Z and SR performed the systematic literature review (SR); MR-C and PB-Z drafted the manuscript. BK supervised the methodology of the SR and the Delphi process. All authors edited the manuscript and accepted its final form.

    • Funding European League Against Rheumatism.

    • Competing interests SB’s salary is part funded by the Birmingham Biomedical Research Centre.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement All data relevant to the study are included in the article.