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Infection and Inflammatory Disorders
Inflammatory and Autoimmune Disorders
IgG4-Related Disease/Hypertrophic Pachymeningitis
IgG4-Related Disease/Hypertrophic Pachymeningitis
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Idiopathic hypertrophic pachymeningitis (IHP)
              • IgG4-related disease
              • IgG4-related hypertrophic pachymeningitis (IgG4-RHP)

            • Definitions

              • Multiorgan immune-mediated condition, which mimics multiple malignant, infectious, and inflammatory disorders
                • 1st defined for pancreatic involvement in 2003
                • May involve pachymeninges of CNS
                  • Chronic progressive diffuse inflammatory fibrosis of dura of brain or spine

            IMAGING

            • General Features

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Musto A et al: Hypertrophic pachymeningitis IgG4-related with antineutrophil cytoplasmic antibody positivity and aortitis. Rheumatol Adv Pract. 3(1):rkz006, 2019
                                  2. De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res. 65(1):356-94, 2016
                                  3. Kamisawa T et al: IgG4-related disease. Lancet. 385(9976):1460-71, 2014
                                  4. Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
                                  5. Mahajan VS et al: IgG4-related disease. Annu Rev Pathol. 9:315-47, 2014
                                  6. Horger M et al: Systemic IgG4-related sclerosing disease: spectrum of imaging findings and differential diagnosis. AJR Am J Roentgenol. 199(3):W276-82, 2012
                                  7. Katsura M et al: Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology. 54(8):873-82, 2012
                                  8. Khosroshahi A et al: A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 23(1):57-66, 2011
                                  9. Chan SK et al: IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 33(8):1249-52, 2009
                                  10. Lowden MR et al: Teaching neuroImage: idiopathic hypertrophic spinal pachymeningitis. Neurology. 72(5):e27, 2009
                                  11. Bosman T et al: Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature. Rheumatol Int. 28(7):713-8, 2008
                                  12. Shobha N et al: Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas. J Clin Neurosci. 15(4):418-27, 2008
                                  13. Lieberman AP: Inflammatory dural masses: if it's not one thing, it's another. J Neuroophthalmol. 27(2):89-90, 2007
                                  14. Pai S et al: Idiopathic hypertrophic spinal pachymeningitis: report of two cases with typical MR imaging findings. AJNR Am J Neuroradiol. 28(3):590-2, 2007
                                  15. Yunokawa K et al: Hypertrophic spinal pachymeningitis associated with heavy-chain disease. Case report. J Neurosurg Spine. 7(4):459-62, 2007
                                  16. Ito Z et al: Recurrence of hypertrophic spinal pachymeningitis. Report of two cases and review of the literature. J Neurosurg Spine. 4(6):509-13, 2006
                                  17. Marangoni S et al: Neurosarcoidosis. Clinical description of 7 cases with a proposal for a new diagnostic strategy. J Neurol. 253(4):488-95, 2006
                                  18. McKinney AM et al: Inflammatory myofibroblastic tumor of the orbit with associated enhancement of the meninges and multiple cranial nerves. AJNR Am J Neuroradiol. 27(10):2217-20, 2006
                                  19. Mehta A et al: Hypertrophic spinal pachymeningitis presenting as compressive myelopathy: a case report. Indian J Pathol Microbiol. 2006 Apr;49(2):286-8. Erratum in: Indian J Pathol Microbiol. 49(3):476, 2006
                                  20. Kazem IA et al: Best cases from the AFIP: idiopathic tumefactive hypertrophic pachymeningitis. Radiographics. 25(4):1075-80, 2005
                                  21. Muthukumar N et al: Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci. 12(5):589-92, 2005
                                  22. D'Andrea G et al: Idiopathic intracranial hypertrophic pachymeningitis: two case reports and review of the literature. Neurosurg Rev. 27(3):199-204, 2004
                                  23. Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
                                  24. Sridhar K et al: Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg. 18(5):515-7, 2004
                                  25. Lee YC et al: Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol. 24(1):119-23, 2003
                                  26. Riku S et al: Idiopathic hypertrophic pachymeningitis. Neuropathology. 23(4):335-44, 2003
                                  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

                                            TERMINOLOGY

                                            • Synonyms

                                              • Idiopathic hypertrophic pachymeningitis (IHP)
                                              • IgG4-related disease
                                              • IgG4-related hypertrophic pachymeningitis (IgG4-RHP)

                                            • Definitions

                                              • Multiorgan immune-mediated condition, which mimics multiple malignant, infectious, and inflammatory disorders
                                                • 1st defined for pancreatic involvement in 2003
                                                • May involve pachymeninges of CNS
                                                  • Chronic progressive diffuse inflammatory fibrosis of dura of brain or spine

                                            IMAGING

                                            • General Features

                                              • MR Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Staging, Grading, & Classification

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Musto A et al: Hypertrophic pachymeningitis IgG4-related with antineutrophil cytoplasmic antibody positivity and aortitis. Rheumatol Adv Pract. 3(1):rkz006, 2019
                                                                  2. De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res. 65(1):356-94, 2016
                                                                  3. Kamisawa T et al: IgG4-related disease. Lancet. 385(9976):1460-71, 2014
                                                                  4. Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
                                                                  5. Mahajan VS et al: IgG4-related disease. Annu Rev Pathol. 9:315-47, 2014
                                                                  6. Horger M et al: Systemic IgG4-related sclerosing disease: spectrum of imaging findings and differential diagnosis. AJR Am J Roentgenol. 199(3):W276-82, 2012
                                                                  7. Katsura M et al: Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology. 54(8):873-82, 2012
                                                                  8. Khosroshahi A et al: A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 23(1):57-66, 2011
                                                                  9. Chan SK et al: IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 33(8):1249-52, 2009
                                                                  10. Lowden MR et al: Teaching neuroImage: idiopathic hypertrophic spinal pachymeningitis. Neurology. 72(5):e27, 2009
                                                                  11. Bosman T et al: Idiopathic hypertrophic cranial pachymeningitis treated by oral methotrexate: a case report and review of literature. Rheumatol Int. 28(7):713-8, 2008
                                                                  12. Shobha N et al: Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas. J Clin Neurosci. 15(4):418-27, 2008
                                                                  13. Lieberman AP: Inflammatory dural masses: if it's not one thing, it's another. J Neuroophthalmol. 27(2):89-90, 2007
                                                                  14. Pai S et al: Idiopathic hypertrophic spinal pachymeningitis: report of two cases with typical MR imaging findings. AJNR Am J Neuroradiol. 28(3):590-2, 2007
                                                                  15. Yunokawa K et al: Hypertrophic spinal pachymeningitis associated with heavy-chain disease. Case report. J Neurosurg Spine. 7(4):459-62, 2007
                                                                  16. Ito Z et al: Recurrence of hypertrophic spinal pachymeningitis. Report of two cases and review of the literature. J Neurosurg Spine. 4(6):509-13, 2006
                                                                  17. Marangoni S et al: Neurosarcoidosis. Clinical description of 7 cases with a proposal for a new diagnostic strategy. J Neurol. 253(4):488-95, 2006
                                                                  18. McKinney AM et al: Inflammatory myofibroblastic tumor of the orbit with associated enhancement of the meninges and multiple cranial nerves. AJNR Am J Neuroradiol. 27(10):2217-20, 2006
                                                                  19. Mehta A et al: Hypertrophic spinal pachymeningitis presenting as compressive myelopathy: a case report. Indian J Pathol Microbiol. 2006 Apr;49(2):286-8. Erratum in: Indian J Pathol Microbiol. 49(3):476, 2006
                                                                  20. Kazem IA et al: Best cases from the AFIP: idiopathic tumefactive hypertrophic pachymeningitis. Radiographics. 25(4):1075-80, 2005
                                                                  21. Muthukumar N et al: Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci. 12(5):589-92, 2005
                                                                  22. D'Andrea G et al: Idiopathic intracranial hypertrophic pachymeningitis: two case reports and review of the literature. Neurosurg Rev. 27(3):199-204, 2004
                                                                  23. Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
                                                                  24. Sridhar K et al: Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg. 18(5):515-7, 2004
                                                                  25. Lee YC et al: Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol. 24(1):119-23, 2003
                                                                  26. Riku S et al: Idiopathic hypertrophic pachymeningitis. Neuropathology. 23(4):335-44, 2003