Large granular lymphocytes (LGL) in primary Sjögren syndrome (pSS): immunophenotype and review on the pathological role of T cells in pSS

摘要

Primary Sjogren syndrome (pSS) is a chronic autoimmune systemic disease that mainly affects the exocrine glands, causing severe inflammation with accompanying destruction of the gland. It is also characterized by systemic symptoms and laboratory findings of polyclonal B-lymphocyte activation, hypergammaglobulinemia, and positive autoantibodies. Its pathophysiology is not yet fully understood; genetic factors seem to play a relatively minor role, while environmental ones, most likely infections, contribute to disease onset and progression by activation of the innate and adaptive immune systems. Although the role of B cells in the disease is better characterized, little is known about the involvement of T cells in pSS [ - ].