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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

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Deshpande V: IgG4 related disease of the head and neck. Head Neck Pathol. 9(1):24-31, 2015
                                      2. Joshi D et al: Cerebral involvement in IgG4-related disease. Clin Med. 15(2):130-4, 2015
                                      3. Khosroshahi A et al: International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. ePub, 2015
                                      4. Tauziede-Espariat A et al: The prevalence of IgG4-positive plasma cells in hypophysitis: a possible relationship to IgG4-related disease. Clin Neuropathol. ePub, 2015
                                      5. Lee YS et al: Immunoglobulin g4-related hypertrophic pachymeningitis with skull involvement. Brain Tumor Res Treat. 2(2):87-91, 2014
                                      6. Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
                                      7. Soussan M et al: IgG4-related diffuse perineural disease. Neurology. 83(20):1877-8, 2014
                                      8. Tiegs-Heiden CA et al: Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 35(7):1393-7, 2014
                                      9. Yamamoto M et al: IgG4 disease. J Neuroophthalmol. 34(4):393-9, 2014
                                      10. 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

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Deshpande V: IgG4 related disease of the head and neck. Head Neck Pathol. 9(1):24-31, 2015
                                                                          2. Joshi D et al: Cerebral involvement in IgG4-related disease. Clin Med. 15(2):130-4, 2015
                                                                          3. Khosroshahi A et al: International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. ePub, 2015
                                                                          4. Tauziede-Espariat A et al: The prevalence of IgG4-positive plasma cells in hypophysitis: a possible relationship to IgG4-related disease. Clin Neuropathol. ePub, 2015
                                                                          5. Lee YS et al: Immunoglobulin g4-related hypertrophic pachymeningitis with skull involvement. Brain Tumor Res Treat. 2(2):87-91, 2014
                                                                          6. Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
                                                                          7. Soussan M et al: IgG4-related diffuse perineural disease. Neurology. 83(20):1877-8, 2014
                                                                          8. Tiegs-Heiden CA et al: Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 35(7):1393-7, 2014
                                                                          9. Yamamoto M et al: IgG4 disease. J Neuroophthalmol. 34(4):393-9, 2014
                                                                          10. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012