Overview of IgG4-Related Kidney Disease

IgG4-related kidney disease (IgG4-RKD) is a comprehensive term for the renal lesions associated with IgG4-related disease(IgG4-RD), including IgG4-related tubulointerstitial nephritis(IgG4-related TIN), glomerular lesions, and IgG4-related pelvic lesion. The unifying disease entity of IgG4-RKD was elucidated after autoimmune pancreatitisand Mikulicz disease were recognized to occur frequently together. Involvement of multiple other organs was also identified to be part of this disease over the first dozen years of this century. A diagnostic algorithm and criteria for IgG4-RKD were proposed and published in 2011 by the IgG4-RKD working group of the Japanese Society of Nephrology. Simultaneously, another set of criteria for IgG4-related TIN was presented in the United States.