Development of targeted therapies in IgG4-related disease

IgG4-related disease (IgG4-RD) is a systemic disease characterized by high serum IgG4 levels, infiltration of lymphocytes and IgG4-positive plasma cells into affected tissues, and subsequent fibrosis, forming mass, nodular, and thickened lesions in organs. Although glucocorticoids (GCs) are the first-line treatment for IgG4-RD, the disease often relapses during dose reduction or after discontinuation of GC. Long-term treatment with GC is associated with adverse effects such as infection, osteoporosis, and atherosclerosis. Therefore, there is an urgent need to develop a treatment strategy that specifically addresses the pathogenesis of IgG4-RD. As immunocompetent cells and immune-related molecules involved in the pathogenesis of IgG4-RD are increasingly being identified, there is a growing demand for new molecular-targeted drugs that target them. In particular, favourable results have been reported for drugs that target B cells, such as anti-cluster of differentiation (CD)20 and anti-CD19 antibodies. In addition, clinical trials are underway for new therapeutic agents, such as anti-signalling lymphocytic activation molecule family 7 antibodies that target T cells and other cells.