Takao Saito, Emeritus Professor, Faculty of Medicine, Fukuoka UniversityJohn H. Stone, Massachusetts General Hospital Hitoshi Nakashima, Division of Nephrology & Rheumatology, Department of Internal Medicine, Fukuoka University Takako Saeki, Department of Internal Medicine, Nagaoka Red Cross Hospital Mitsuhiro Kawano, Division of Rheumatology, Department of Internal Medicine, Kanazawa University

• 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 kKidneyDiseases 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.

Takao Saito, Emeritus Professor, Faculty of Medicine, Fukuoka UniversityJohn H. Stone, Massachusetts General Hospital Hitoshi Nakashima, Division of Nephrology & Rheumatology, Department of Internal Medicine, Fukuoka University Takako Saeki, Department of Internal Medicine, Nagaoka Red Cross Hospital Mitsuhiro Kawano, Division of Rheumatology, Department of Internal Medicine, Kanazawa University

• 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 kKidneyDiseases 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.

Takao Saito, Emeritus Professor, Faculty of Medicine, Fukuoka UniversityJohn H. Stone, Massachusetts General Hospital Hitoshi Nakashima, Division of Nephrology & Rheumatology, Department of Internal Medicine, Fukuoka University Takako Saeki, Department of Internal Medicine, Nagaoka Red Cross Hospital Mitsuhiro Kawano, Division of Rheumatology, Department of Internal Medicine, Kanazawa University

• 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 kKidneyDiseases 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.