Sjögren Disease

In 1933, the Swedish ophthalmologist Hendrik Sjögren was the first to suggest that behind the dryness that several of his patients presented, there could be a systemic disease related to an abnormal immune response. Since then, the term Sjögren’s disease (SjS) has been used and it has been considered a minor and infrequent disorder compared to other systemic autoimmune diseases (SAD) and, consequently, with little progress both in clinical and therapeutic research. However, despite SjS causing dysfunction of the exocrine secretory glands with consequent dryness of the main mucosal surfaces this disease can also affect internal organs and increase the risk of developing hematological malignancies. Criteria have been proposed for the correct classification of patients and are based on the main diagnostic tools for SjS diagnosis. Herein, after a brief description of SjS pathophysiology, these criteria are extensively discussed.