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
Musto A et al: Hypertrophic pachymeningitis IgG4-related with antineutrophil cytoplasmic antibody positivity and aortitis. Rheumatol Adv Pract. 3(1):rkz006, 2019
De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res. 65(1):356-94, 2016
Kamisawa T et al: IgG4-related disease. Lancet. 385(9976):1460-71, 2014
Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
Mahajan VS et al: IgG4-related disease. Annu Rev Pathol. 9:315-47, 2014
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
Katsura M et al: Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology. 54(8):873-82, 2012
Khosroshahi A et al: A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 23(1):57-66, 2011
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
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
Shobha N et al: Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas. J Clin Neurosci. 15(4):418-27, 2008
Lieberman AP: Inflammatory dural masses: if it's not one thing, it's another. J Neuroophthalmol. 27(2):89-90, 2007
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
Yunokawa K et al: Hypertrophic spinal pachymeningitis associated with heavy-chain disease. Case report. J Neurosurg Spine. 7(4):459-62, 2007
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
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
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
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
Kazem IA et al: Best cases from the AFIP: idiopathic tumefactive hypertrophic pachymeningitis. Radiographics. 25(4):1075-80, 2005
Muthukumar N et al: Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci. 12(5):589-92, 2005
D'Andrea G et al: Idiopathic intracranial hypertrophic pachymeningitis: two case reports and review of the literature. Neurosurg Rev. 27(3):199-204, 2004
Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
Sridhar K et al: Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg. 18(5):515-7, 2004
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
Riku S et al: Idiopathic hypertrophic pachymeningitis. Neuropathology. 23(4):335-44, 2003
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
Musto A et al: Hypertrophic pachymeningitis IgG4-related with antineutrophil cytoplasmic antibody positivity and aortitis. Rheumatol Adv Pract. 3(1):rkz006, 2019
De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res. 65(1):356-94, 2016
Kamisawa T et al: IgG4-related disease. Lancet. 385(9976):1460-71, 2014
Lu LX et al: IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 71(6):785-93, 2014
Mahajan VS et al: IgG4-related disease. Annu Rev Pathol. 9:315-47, 2014
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
Katsura M et al: Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology. 54(8):873-82, 2012
Khosroshahi A et al: A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 23(1):57-66, 2011
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
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
Shobha N et al: Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas. J Clin Neurosci. 15(4):418-27, 2008
Lieberman AP: Inflammatory dural masses: if it's not one thing, it's another. J Neuroophthalmol. 27(2):89-90, 2007
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
Yunokawa K et al: Hypertrophic spinal pachymeningitis associated with heavy-chain disease. Case report. J Neurosurg Spine. 7(4):459-62, 2007
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
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
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
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
Kazem IA et al: Best cases from the AFIP: idiopathic tumefactive hypertrophic pachymeningitis. Radiographics. 25(4):1075-80, 2005
Muthukumar N et al: Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci. 12(5):589-92, 2005
D'Andrea G et al: Idiopathic intracranial hypertrophic pachymeningitis: two case reports and review of the literature. Neurosurg Rev. 27(3):199-204, 2004
Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
Sridhar K et al: Idiopathic chronic hypertrophic pachymeningitis causing thoracic cord compression. Br J Neurosurg. 18(5):515-7, 2004
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
Riku S et al: Idiopathic hypertrophic pachymeningitis. Neuropathology. 23(4):335-44, 2003
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