LAUSR

Purpose of review IgG4-related disease (IgG4-RD) is an autoimmune fibrosing condition which is being increasingly recognized. This condition has been classically managed with glucocorticoids (GC) with variable dosing and frequent relapses. Multiple observational reports studied different GC doses and steroid-sparing agents. Rituximab (RTX) was reported as an effective induction treatment, with less relapses after 1 year of treatment. In the absence of guidelines, an international management consensus was made in 2015. Recent findings In the last few years, significant advances have been made to clarify IgG4-RD pathophysiology. The new classification criteria will enable investigators to design new clinical trials with more homogeneous populations. Furthermore, B and T lymphocytes have a central role in the inflammatory cascade. More solid evidence has been published supporting treatment with GC, RTX, and some other steroid-sparing agents like mofetil mycophenolate. The combination of GC and immunosuppressants has been pointed by a meta-analysis as the most effective treatment for IgG4-RD, although the number and quality of studies remains limited. Summary The use of GC and steroid-sparing agents is effective in IgG4-RD, although the choice between rituximab and other immunosuppressants can be influenced by many factors. Novel agents will be trialed in the following years.