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IgG4 Renal Disease
Kevin Kalisz, MD; Benjamin Wildman-Tobriner, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Immunoglobulin G4-related disease (IgG4-RD)
              • Definitions

                • Renal lesions or histopathologic involvement in setting of IgG4-RD
              • Associated Syndromes

                • IgG4 disease involvement in other organs

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            • Reporting Tips

                                              Selected References

                                              1. Kamo M et al: CT findings of upper urinary tract lesions in IgG4-related disease: comparison with urothelial carcinoma. AJR Am J Roentgenol. 215(2):406-12, 2020
                                              2. Oh JW et al: Immunoglobulin G4-related disease of the genitourinary system: spectrum of imaging findings and clinical-pathologic features. Radiographics. 40(5):1265-83, 2020
                                              3. Evans RDR et al: Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort. Kidney Int Rep. 4(1):48-58, 2019
                                              4. Salvadori M et al: Immunoglobulin G4-related kidney diseases: an updated review. World J Nephrol. 7(1):29-40, 2018
                                              5. Haldar D et al: An overview of the diagnosis and management of immunoglobulin G4-related disease. CMAJ. 188(13):953-61, 2016
                                              6. Mizushima I et al: Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease: a retrospective multicenter study. Arthritis Res Ther. 18(1):273, 2016
                                              7. Carruthers MN et al: Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 74(6):1171-7, 2015
                                              8. Seo N et al: Immunoglobulin G4-related kidney disease: a comprehensive pictorial review of the imaging spectrum, mimickers, and clinicopathological characteristics. Korean J Radiol. 16(5):1056-67, 2015
                                              9. Kim B et al: IgG4-related kidney disease: MRI findings with emphasis on the usefulness of diffusion-weighted imaging. Eur J Radiol. 83(7):1057-62, 2014
                                              10. Raissian Y et al: Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 22(7):1343-52, 2011
                                              11. Saeki T et al: Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 78(10):1016-23, 2010
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Immunoglobulin G4-related disease (IgG4-RD)
                                                          • Definitions

                                                            • Renal lesions or histopathologic involvement in setting of IgG4-RD
                                                          • Associated Syndromes

                                                            • IgG4 disease involvement in other organs

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      • Image Interpretation Pearls

                                                                                        • Reporting Tips

                                                                                          Selected References

                                                                                          1. Kamo M et al: CT findings of upper urinary tract lesions in IgG4-related disease: comparison with urothelial carcinoma. AJR Am J Roentgenol. 215(2):406-12, 2020
                                                                                          2. Oh JW et al: Immunoglobulin G4-related disease of the genitourinary system: spectrum of imaging findings and clinical-pathologic features. Radiographics. 40(5):1265-83, 2020
                                                                                          3. Evans RDR et al: Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort. Kidney Int Rep. 4(1):48-58, 2019
                                                                                          4. Salvadori M et al: Immunoglobulin G4-related kidney diseases: an updated review. World J Nephrol. 7(1):29-40, 2018
                                                                                          5. Haldar D et al: An overview of the diagnosis and management of immunoglobulin G4-related disease. CMAJ. 188(13):953-61, 2016
                                                                                          6. Mizushima I et al: Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease: a retrospective multicenter study. Arthritis Res Ther. 18(1):273, 2016
                                                                                          7. Carruthers MN et al: Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 74(6):1171-7, 2015
                                                                                          8. Seo N et al: Immunoglobulin G4-related kidney disease: a comprehensive pictorial review of the imaging spectrum, mimickers, and clinicopathological characteristics. Korean J Radiol. 16(5):1056-67, 2015
                                                                                          9. Kim B et al: IgG4-related kidney disease: MRI findings with emphasis on the usefulness of diffusion-weighted imaging. Eur J Radiol. 83(7):1057-62, 2014
                                                                                          10. Raissian Y et al: Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 22(7):1343-52, 2011
                                                                                          11. Saeki T et al: Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 78(10):1016-23, 2010