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IgG4-Related Disease in Head & Neck
Carmen C. Cho, MBBS, FRCR; Evelyn W. K. Tang, MBBS, FRCR; Anil T. Ahuja, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

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

            • Previously recognized head and neck conditions now acknowledged to fall within spectrum of IgG4-RD
              • Mikulicz syndrome
              • Küttner tumor
              • Riedel thyroiditis
              • Inflammatory pseudotumor
              • Eosinophilic angiocentric fibrosis
          • Definitions

            • Recently recognized idiopathic systemic fibroinflammatory condition characterized by tendency to form tumor-like lesion with dense lymphoplasmacytic infiltrate rich in IgG4-positive cells

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            • Immunohistochemical Features

                              • Serum IgG4 Level

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Thompson A et al: Imaging of IgG4-related disease of the head and neck. Clin Radiol. ePub, 2017
                                            2. Mulholland GB et al: Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg. 44:24, 2015
                                            3. Ferry JA et al: IgG4-related disease in the head and neck. Semin Diagn Pathol. 29(4):235-44, 2012
                                            4. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
                                            5. Inoue D et al: IgG4-related perineural disease. Int J Rheumatol. 2012:401890, 2012
                                            6. Stone JH et al: Mechanisms of disease: IgG4-related disease. N Engl J Med. 36:539–51, 2012
                                            7. Stone JH: IgG4-related disease: nomenclature, clinical features, and treatment. Semin Diagn Pathol. 29(4):177-90, 2012
                                            8. Toyoda K et al: MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol. 33(11):2136-9, 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

                                                    TERMINOLOGY

                                                    • Abbreviations

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

                                                      • Previously recognized head and neck conditions now acknowledged to fall within spectrum of IgG4-RD
                                                        • Mikulicz syndrome
                                                        • Küttner tumor
                                                        • Riedel thyroiditis
                                                        • Inflammatory pseudotumor
                                                        • Eosinophilic angiocentric fibrosis
                                                    • Definitions

                                                      • Recently recognized idiopathic systemic fibroinflammatory condition characterized by tendency to form tumor-like lesion with dense lymphoplasmacytic infiltrate rich in IgG4-positive cells

                                                    IMAGING

                                                    • General Features

                                                      • Ultrasonographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Microscopic Features

                                                                      • Immunohistochemical Features

                                                                        • Serum IgG4 Level

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Thompson A et al: Imaging of IgG4-related disease of the head and neck. Clin Radiol. ePub, 2017
                                                                                      2. Mulholland GB et al: Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg. 44:24, 2015
                                                                                      3. Ferry JA et al: IgG4-related disease in the head and neck. Semin Diagn Pathol. 29(4):235-44, 2012
                                                                                      4. Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
                                                                                      5. Inoue D et al: IgG4-related perineural disease. Int J Rheumatol. 2012:401890, 2012
                                                                                      6. Stone JH et al: Mechanisms of disease: IgG4-related disease. N Engl J Med. 36:539–51, 2012
                                                                                      7. Stone JH: IgG4-related disease: nomenclature, clinical features, and treatment. Semin Diagn Pathol. 29(4):177-90, 2012
                                                                                      8. Toyoda K et al: MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol. 33(11):2136-9, 2012