Part I. General Remarks -- 1. IgG4-Related Disease: History and Overview -- 2. IgG4-Related Disease: Pathological Features and Differential Diagnosis -- 3. Potential Pathways in The Pathogenesis of IgG4-Related Disease -- 4. Does IgG4-Related Disease Have an Autoimmune Basis? -- 5. Plasmablasts: A Promising Biomarker in IgG4-RD -- Part II. Features of IgG4-Related Ridney Disease and Associated Conditions -- 6. Overview of IgG4-Related Kidney Disease -- 7. IgG4-Related Kidney Disease: Clinical and Laboratory Features -- 8. Imaging Findings -- 9. Characteristic Tubulointerstitial Nephritis in IgG4-Related Kidney Disease -- 10. Electron Microscopic Findings -- 11. IgG4-RelatedKidney Disease: Pathological Features �Membranous Glomerulonephritis -- 12. IgG4-RelatedKidney Disease: Pathological Features �Other Glomerular Disease -- 13. IgG4-Related kKidney Diseases and Conditions: Renal Pelvic and Ureteral Diseases -- 14. Retroperitoneal Fibrosis/Periaortitis and Hydronephrosis -- 15. Other Urogenital Lesions (Testis, Paratesticular Lesion, Bladder, Prostate etc.) -- 16. Characteristic Distribution of Inflammatory Lesions in IgG4-Related Kidney Disease: Findings from Autopsy Case Series -- 17. Diagnosis of IgG4-Related Kidney Disease -- 18. Pathogenesis of IgG4-Related Kidney Disease -- Part III. Treatment of IgG4-RD and IgG4-RKD -- 19. Corticosteroid Therapy for IgG4-Related Kidney Disease -- 20. Targeting the B Cell Lineage in IgG4-Related Disease -- Part IV. Differential Diagnosis of IgG4-RKD -- 21. Differential Diagnosis of IgG4-Related Tubulointerstitial Nephritis �An Overview -- 22. Differential Diagnosis (1): ANCA Associated Vasculitis -- 23. Differential Diagnosis (2): Castleman Disease -- 24. Differential Diagnosis (3): Sjögren Syndrome -- 25. Differential Diagnosis: Lupus Nephritis and IgG4-Related Kidney Disease -- Part V. Lessons from Cases -- 26. Multicentric Castleman�s Disease Mimicking IgG4-Related Disease; A Case Report -- 27. A Case of Concurrent IgG4-Related Kidney Disease and Lupus Nephritis.

This book offers a detailed review of the pathological and imaging features, diagnosis and treatment of IgG4-Related Kidney Disease (IgG4-RKD). IgG4-related disease (IgG4-RD), 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 IgG4-RD have been reported and are recently attracting attention as IgG4-RKD. In this book, leading researchers in the field present the latest insights into the broad spectrum of IgG4-RKD characteristics. In addition, they provide a detailed explanation of the pathology of IgG4-RKD including comparisons between the kidney and other affected organs, such as the lacrimal, salivary glands and pancreas in the histopathological section. IgG4-Related Kidney Disease will have a major impact on future immunological and nephrological studies and offers a useful resource not only for nephrologists but also general physicians and investigators in related fields.