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IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz’s disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity.

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Despite similarities in the involved organs, there are considerable clinical and pathological differences between IgG(4)+MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG(4)+MOLPS.

Aim-To evaluate the eYcacy of autologous serum application for the treatment of dry eye in Sjögren's syndrome. Methods-The stability of essential components (EGF, vitamin A, and TGF-) in preserved serum were examined following preservation at 4°C and −20°C. In a primary clinical trial, 12 patients with Sjögren's syndrome were treated with autologous serum (diluted to 20% with sterile saline) for 4 weeks, and vital staining of the ocular surface was compared before and after treatment. The eVects of serum on mucin (MUC-1) expression were observed in cultured conjunctival epithelial cells in vitro. Results-EGF, vitamin A, and TGFwere well preserved for up to 1 month in the refrigerator at 4°C and up to 3 months in the freezer at −20°C. Rose bengal and fluorescein scores improved significantly from the initial scores of 5.3 and 5.6 to 1.7 and 2.5 after 4 weeks, respectively. The additive eVect of human serum for cultured conjunctival epithelial cells showed significant MUC-1 upregulation on the cell surface. Conclusion-Autologous serum application is a safe and eYcient way to provide essential components to the ocular surface in the treatment of dry eye associated with Sjögren's syndrome. (Br J Ophthalmol 1999;83:390-395)