TY - JOUR T1 - Primary versus Secondary Sjögren Syndrome: Is It Time To Reconsider These Terms? JF - The Journal of Rheumatology JO - J Rheumatol SP - 665 LP - 666 DO - 10.3899/jrheum.180392 VL - 46 IS - 7 AU - CLIO P. MAVRAGANI AU - HARALAMPOS M. MOUTSOPOULOS Y1 - 2019/07/01 UR - http://www.jrheum.org/content/46/7/665.abstract N2 - Classification of systemic autoimmune diseases on the basis of clinical, serological, and genetic characteristics has long been considered a necessary approach to determine disease prognosis and institute appropriate therapeutic strategies1.Has this, however, truly been pursued by the scientific community? Probably not, because similar disease-modifying agents and several immunosuppressants are implemented as baseline therapy for combating arthritis, pleurisy, or nephritis, irrespective of the character of the underlying disease. Despite our better understanding of molecular patho-biology, which eventually led to development of targeted therapies, seropositive and seronegative arthritis are still similarly treated with tumor necrosis factor inhibitors, while certain vasculitic syndromes are managed with B cell depletion therapy in the same way as rheumatoid arthritis (RA). The responses, even to those new therapies, are not uniform because these syndromes are highly polymorphic in both clinical phenotypes and underlying pathogenetic pathways.A prototype autoimmune disorder characterized by a wide spectrum of clinical, serological, and genetic features is Sjögren syndrome (SS). It may extend from sicca symptoms and complications of mucosal dryness as a result of exocrine gland involvement, to a systemic disease or to malignant B cell lymphoproliferation in about one-fourth and one-tenth of patients, respectively. Nevertheless, sicca manifestations may also occur in several other systemic autoimmune disorders at variable frequencies ranging from 8.3% … Address correspondence to Dr. C.P. Mavaragani, Department of Physiology, 75 Mikras Asias St., 11527 Athens, Greece. E-mail: kmauragan{at}med.uoa.gr ER -