Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations

Pilar Brito-Zeron, Elke Theander, Chiara Baldini, Raphaele Seror, Soledad Retamozo, Luca Quartuccio, Hendrika Bootsma, Simon J. Bowman, Thomas Doerner, Jacques-Eric Gottenberg, Xavier Mariette, Stefano Bombardieri, Salvatore de Vita, Thomas Mandl, Wan-Fai Ng, Aike A. Kruize, Athanasios Tzioufas, Claudio Vitali, Jill Buyon, Peter IzmirlyRobert Fox, Manuel Ramos-Casals*, EULAR Sjogren Syndrome Task Force

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

62 Citations (Scopus)

Abstract

Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.

Original languageEnglish
Pages (from-to)137-156
Number of pages20
JournalExpert review of clinical immunology
Volume12
Issue number2
DOIs
Publication statusPublished - 1-Feb-2016

Keywords

  • systemic activity
  • classification criteria
  • Sjogren syndrome
  • early diagnosis
  • ESSDAI
  • anti-Ro/SS-A antibodies
  • focal lymphocytic sialadenitis
  • SALIVARY-GLAND ULTRASONOGRAPHY
  • RHEUMATOLOGY CLASSIFICATION CRITERIA
  • EUROPEAN CONSENSUS GROUP
  • THROMBOTIC THROMBOCYTOPENIC PURPURA
  • PROGRESSIVE MULTIPLE-SCLEROSIS
  • INTERSTITIAL LUNG-DISEASE
  • PATIENT-REPORTED INDEX
  • OF-THE-LITERATURE
  • NEUROMYELITIS-OPTICA
  • PERIPHERAL NEUROPATHIES

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