Christelle Le Dantec,1 Amandine Charras,1 Anne Bordron,1 Wesley H. Brooks,2 *Yves Renaudineau1,3
1. INSERM ESPRI, ERI29/EA2216, LabEx IGO “Immunotherapy Graft Oncology”, Innovative Medicines Initiative PRECISESADS, Réseau épigénétique et réseau canaux ioniques du Cancéropole Grand Ouest, European University of Brittany, Brest, France
2. Department of Chemistry, University of South Florida, Tampa, Florida, USA
3. Laboratory of Immunology and Immunotherapy, Brest University Medical School, Hôpital Morvan, Brest, France
*Correspondence to firstname.lastname@example.org
Disclosure: The authors have declared no conflicts of interest.
Received: 03.03.16 Accepted: 11.07.16
Citation: EMJ. 2016;1:21-28.
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune epithelitis and recent advances in our comprehension of its pathophysiology strongly suggest a multi-step process that involves environmental factors (e.g. chronic viral infection, drugs), followed by deregulation of the epigenetic machinery (e.g. DNA demethylation, histone modifications, microRNAs), which in turn specifically affects lymphocytes and epithelial cells leading to an aberrant inflammation. This process is amplified in the case of genetic mutations. As a consequence, autoreactive lymphocytes and autoantigens are produced leading to the development of autoantibodies. Moreover, it was observed that epigenetic modifications in pSS could be reversed, thus providing arguments to suggest that therapeutic strategies targeting the epigenetic deregulation and in particular the PKC-delta/Erk/DNMT1 pathway would be effective in pSS.