IgG4-Related Disease: A Concise Review

Abstract

IgG4-related disease (IgG4-RD) is a multi-organ fibroinflammatory disorder that may result in organ dysfunction and complications. Glucocorticoids remain the primary treatment for inducing remission, while recently reported disease phenotypes might inform personalized therapeutic strategies. Progress in understanding the pathophysiology of IgG4-RD has led to the identification of novel treatment targets. Ongoing research will further refine the optimal treatment for IgG4-RD. This review offers an update on the latest evidence on the epidemiology, pathogenesis, clinical features, diagnosis, and treatment of IgG4-RD.