Update on IgA nephropathy: implications for treatment in IgA vasculitis: a guide for rheumatologists

IgA vasculitis (IgAV) and IgA nephropathy (IgAN) have historically been treated as distinct disease entities in clinical practice and in drug development, despite growing evidence speaking to a shared pathogenesis. Recent years have seen an explosion in therapeutic trials in the IgAN space, with multiple approvals of targeted therapies and a shifting paradigm of care; meanwhile, IgAV has lagged behind with a paucity of randomized trials. What can rheumatologists learn from IgAN, and can IgAV patients benefit from therapeutic advances in IgAN?

In this article, we will compare and contrast the epidemiology and clinical presentations of IgAV and IgAN, and explore the evidence supporting a shared pathogenesis including the 'four hit hypothesis'. We will review emerging therapies in IgAN and their biologic basis for the rheumatology audience, and discuss how lessons learned in IgAN might be applied to IgAV.

Rheumatologists should be aware of the therapeutic advances in IgAN, which may inform future management of IgAV patients, particularly those with renal involvement.