IgG4-related disease (IgG4-RD) is an emerging condition highly relevant to rheumatologists because of its immune-mediated nature, its propensity to cause multi-organ disease, and its ability to mimic multiple classic rheumatologic conditions. IgG4-RD readily mimics Sjogren's syndrome, lupus nephritis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, sarcoidosis, large-vessel vasculitis (e.g., giant cell arteritis) and others. Despite its only recent recognition approximately a dozen years ago, we have learned much about the pathophysiology of this condition. Our understanding of disease mechanisms informs both current and future approaches to treatment. I will discuss the practical, hands-on management of IgG4-RD and also consider ways in which our mechanistic understanding is driving current treatment trials.
Disclosure of Interest J. Stone Grant/research support from: Roche, Genentech, Xencor, Consultant for: Roche, Genentech, Xencor