musculoskeletal and connective tissue disorders
IgG4 Related Disease
Treatment of IgG4 Related Disease
Corticosteroids B-cell targeted therapy withrituximab Treatment of IgG4-RD aims to reduce inflammation, induce remission, and preserve organ function. Typically, tumor-like masses or organ enlargement should normalize following treatment. When organs fail to normalize in response to 1 to 2 months of high-dose corticosteroids or 3 to 4 months following B-cell-depleting therapy, this usually indicates irreversible fibrosis, which is common in patients with retroperitoneal fibrosis, longstanding organ involvement, or an incorrect diagnosis. is nearly universally effective in treating active IgG4-RD, but randomized trials are lacking ( Treatment reference ). Some patients require surgical procedures, such as stenting, to relieve mechanical obstruction of the ureters or bile ducts. Serum IgG4 levels generally decline with treatment over the course of months but often never normalize. Khosroshahi A, Wallace ZS, Crowe JL, et al: International consensus guidance statement on the management and treatment of IgG4-related disease.Arthritis Rheumatol67(7):1688-99, 2015. doi: 10.1002/art.39132 