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IgG4-Related Disease
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • IgG4-related disease (IgG4-RD)
            • Synonyms

              • Organ-specific conditions now related to IgG4
                • Mikulicz disease (lacrimal, parotid, submandibular glands)
                • Autoimmune pancreatitis (AIP)
                • Ormond disease (retroperitoneal fibrosis)
                • IgG4-related ophthalmic disease
              • "Idiopathic" pseudotumor (generic)
            • Definitions

              • Chronic fibroinflammatory disease characterized by IgG4(+) plasma cell infiltrates

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross, Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Peng L et al: Clinical features of immunoglobulin G4-related disease with central nervous system involvement: an analysis of 15 cases. Clin Exp Rheumatol. ePub, 2020
                                      2. Satou A et al: Clinicopathological differential diagnosis of IgG4-related disease: a historical overview and a proposal of the criteria for excluding mimickers of IgG4-related disease. Pathol Int. ePub, 2020
                                      3. Vakrakou AG et al: Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review. Rheumatol Int. 40(2):337-43, 2020
                                      4. Goulam-Houssein S et al: IgG4-related intracranial disease. Neuroradiol J. 32(1):29-35, 2019
                                      5. Kuroda N et al: Intracranial multiple pseudotumor due to immunoglobulin G4-related disease without other lesions: case report and literature review. World Neurosurg. 132:69-74, 2019
                                      6. Ryu G et al: Clinical significance of IgG4 in sinonasal and skull base inflammatory pseudotumor. Eur Arch Otorhinolaryngol. ePub, 2019
                                      7. AbdelRazek MA et al: IgG4-related disease of the central and peripheral nervous systems. Lancet Neurol. 17(2):183-92, 2018
                                      8. Tanji H et al: Inflammatory Pseudotumor of the Brain Parenchyma with IgG4 Hypergammaglobulinemia. Intern Med. 55(14):1911-6, 2016
                                      9. Deshpande V: IgG4 related disease of the head and neck. Head Neck Pathol. 9(1):24-31, 2015
                                      10. Okano A et al: Intracranial Inflammatory Pseudotumors Associated with Immunoglobulin G4-Related Disease Mimicking Multiple Meningiomas: A Case Report and Review of the Literature. World Neurosurg. 83(6):1181.e1-4, 2015
                                      11. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • IgG4-related disease (IgG4-RD)
                                                • Synonyms

                                                  • Organ-specific conditions now related to IgG4
                                                    • Mikulicz disease (lacrimal, parotid, submandibular glands)
                                                    • Autoimmune pancreatitis (AIP)
                                                    • Ormond disease (retroperitoneal fibrosis)
                                                    • IgG4-related ophthalmic disease
                                                  • "Idiopathic" pseudotumor (generic)
                                                • Definitions

                                                  • Chronic fibroinflammatory disease characterized by IgG4(+) plasma cell infiltrates

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Nuclear Medicine Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross, Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Peng L et al: Clinical features of immunoglobulin G4-related disease with central nervous system involvement: an analysis of 15 cases. Clin Exp Rheumatol. ePub, 2020
                                                                          2. Satou A et al: Clinicopathological differential diagnosis of IgG4-related disease: a historical overview and a proposal of the criteria for excluding mimickers of IgG4-related disease. Pathol Int. ePub, 2020
                                                                          3. Vakrakou AG et al: Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review. Rheumatol Int. 40(2):337-43, 2020
                                                                          4. Goulam-Houssein S et al: IgG4-related intracranial disease. Neuroradiol J. 32(1):29-35, 2019
                                                                          5. Kuroda N et al: Intracranial multiple pseudotumor due to immunoglobulin G4-related disease without other lesions: case report and literature review. World Neurosurg. 132:69-74, 2019
                                                                          6. Ryu G et al: Clinical significance of IgG4 in sinonasal and skull base inflammatory pseudotumor. Eur Arch Otorhinolaryngol. ePub, 2019
                                                                          7. AbdelRazek MA et al: IgG4-related disease of the central and peripheral nervous systems. Lancet Neurol. 17(2):183-92, 2018
                                                                          8. Tanji H et al: Inflammatory Pseudotumor of the Brain Parenchyma with IgG4 Hypergammaglobulinemia. Intern Med. 55(14):1911-6, 2016
                                                                          9. Deshpande V: IgG4 related disease of the head and neck. Head Neck Pathol. 9(1):24-31, 2015
                                                                          10. Okano A et al: Intracranial Inflammatory Pseudotumors Associated with Immunoglobulin G4-Related Disease Mimicking Multiple Meningiomas: A Case Report and Review of the Literature. World Neurosurg. 83(6):1181.e1-4, 2015
                                                                          11. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012