HARMONIzation and integrative analysis of regional, national and international Cohorts on primary Sjögren’s Syndrome (pSS) towards improved stratification, treatment and health policy making
pSS is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and dry eye and mouth manifestations, systemic involvement and an increased risk of evolution into malignant lymphoma, which is the main disease complication. It affects primarily women (9 females/ 1 male) after menopause. Although the cause of pSS is unknown, the disease develops under the influence of a combination of genetic, environmental, and immune factors, as is the case with other autoimmune disorders. Importantly, pSS is relevant not only due to its clinical impact but also as one of the few “model” diseases to link autoimmunity, cancer development (lymphoproliferation) and the pathogenetic role of infection. Thus, the study of pSS can facilitate research in many areas of medicine; for this reason, the possibility for sustainability and expandability of the platform is enhanced. In addition, pSS has a significant impact on the healthcare systems, similar to that of rheumatoid arthritis. The harmonization, semantic interlinking and the subsequent integration of pSS cohorts foreseen in the HarmonicSS project is highly achievable, based on already shared and internationally-accepted classification criteria and measures of disease activity and damage.