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 pancreatitis and 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.
The proportion of IgG4-RKD in biopsy-proven cases is 0.67 % and its distribution does not show any regional differences in Japan. The most common histological feature of IgG4-RKD is tubulointerstitial nephritis, where the combination of IgG4-positive plasma cells and fiber bundles forms a characteristic fibrosis called “storiform” or “bird’s eye”. Glomerular lesions, most of which are membranous nephropathy, are observed in approximately 30 % of IgG4-RKD cases. In addition to the kidney parenchymal and pelvic lesions, tumefactive lesions can be located around the renal capsule and in the ureteral space and retroperitoneum. Accordingly, IgG4-RKD is closely associated with IgG4-related urological disease.
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