TY - JOUR T1 - IgG4 Syndrome: Old Disease, New Perspective JF - The Journal of Rheumatology JO - J Rheumatol SP - 1369 LP - 1370 DO - 10.3899/jrheum.100383 VL - 37 IS - 7 AU - GEORGE E. FRAGOULIS AU - HARALAMPOS M. MOUTSOPOULOS Y1 - 2010/07/01 UR - http://www.jrheum.org/content/37/7/1369.abstract N2 - In the last few decades, the evolution of biomedical sciences has contributed to a better understanding of the pathogenesis of human disease, the description of new clinical entities, sorting out of disease subgroups, and development of targeted therapeutic interventions1.A new syndrome was identified recently, called IgG4 syndrome. Many terms such as “IgG4-related systemic sclerosing disease,” “IgG4-related autoimmune disease,” “IgG4-related systemic disease,” “IgG4-positive multiorgan lymphoproliferative syndrome,” and others have been used to identify the disease.IgG4 syndrome has various clinical manifestations, such as sclerosing pancreatitis, sclerosing cholangitis, prostatitis, tubulointerstitial nephritis, interstitial pneumonia, and enlargement of salivary glands. In previous years, these clinical entities appeared to be unrelated. Recent studies, however, revealed that they share some common denominators, such as elevated serum IgG4 levels and tissue infiltration by IgG4-positive plasma cells, accompanied by tissue fibrosis and sclerosis2. These serological and histopathological features are considered hallmarks for diagnosis of IgG4 syndrome. Thus, the enlargement of salivary glands, accompanied by high serum IgG4 concentrations and IgG4-positive plasma cells in the infiltrates of the salivary glands, previously called … Address correspondence to Dr. Moutsopoulos. E-mail: hmoutsop{at}med.uoa.gr ER -