This book offers a detailed review of the pathological and imaging features, diagnosis and treatment of IgG4-Related Kidney Disease (IgG4RKD). IgG4-related disease (IgG4RD), which is characterized by an elevated serum IgG4 level and infiltration of systemic organs by IgG4 positive plasma cells, is a newly recognized systemic disease. Diverse renal manifestations including specific tubulointerstitial nephritis, membranous nephropathy, and tumor-like urological diseases extending to the pelvis and urinary tract in IgG4RD have been reported and are recently attracting attention as IgG4RKD. In...