Equal rights in autoimmunity: is Sjögren`s syndrome ever ‘secondary’?

Research output: Contribution to journalReview articlepeer-review


Colleges, School and Institutes


Sjögren’s syndrome (SjS) accompanied by other systemic autoimmune rheumatic connective tissue diseases has historically been termed ‘secondary’ in contrast to ‘primary’ SjS as a standalone entity. However it is a matter of a long-standing debate whether the prefixes ‘primary’ and ‘secondary’, notably including a temporal component, are obsolete in the terminology of SjS. We review the history and the pathophysiological, chronological, genetic, histological and clinical data underlying the concept of ‘secondary’ SjS. There are important unintended consequences of the nomenclature; notably ‘secondary’ SjS has been much less researched and is often excluded from clinical trials. We argue for further research, a change in terminology and more stringent classification. Further we highlight possible opportunities for trials in SjS and other systemic autoimmune diseases that might contribute to an advance in care for all patients with SjS.


Original languageEnglish
Article numberkeaa009
Publication statusPublished - 5 Feb 2020


  • Sjögren’s syndrome, RA, SLE, systemic sclerosis, clinical trials, anti-Ro antibodies, genetics, histology, salivary glands