Therapeutic potential of the interleukin-4/interleukin-13 inhibitor dupilumab for treating IgG4-related disease.

Abstract

Dear Editor, IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory condition with diffuse or focal organ mass formation and shows storiform fibrosis with the infiltration of IgG4-positive plasma cells [1]. Corticosteroids as first-line treatment are effective against IgG4-RD; however, their long-term use may cause serious adverse effects. Dupilumab, a human monoclonal antibody against the IL-4 receptor α-subunit (IL-4Rα), inhibits the signal transduction of IL-4 and IL-13. Thus, dupilumab has been recently approved for treating type 2 inflammatory diseases, such as chronic rhinosinusitis, asthma and atopic dermatitis. We have also recently reported the successful treatment by dupilumab in two cases with severe atopic keratoconjunctivitis, suggesting dupilumab may be a potential alternative agent for another type 2 inflammatory disease—severe ocular allergic disease [2]. Although the pathogenetic mechanisms of IgG4-RD have not been fully uncovered, both innate and adaptive immune responses may be involved in IgG4-RD immunopathogenesis. IL-4 and...