Purpose of review: IgG4-related disease (IgG4-RD) is a recently described fibroinflammatory condition that can affect nearly any organ. Our knowledge of this condition--its manifestations, diagnosis, pathogenesis and treatment--is expanding rapidly.
Recent findings: This review highlights the most recent developments in the field. Particular highlights include new manifestations of the disease, studies on the test characteristics of serum IgG4 concentrations in the diagnosis of IgG4-RD, descriptions of the lack of specificity of IgG4+ plasma cells in histopathological samples and preliminary research on unique B and T lymphocyte populations in the disease.
Summary: Future studies might focus on identifying biomarkers to assist with noninvasive diagnosis of IgG4-RD and conducting trials of novel treatments that might supplant glucocorticoids as the treatment of choice.