Nonspecific, nonneoplastic benign inflammatory lesion without identifiable local or systemic causes characterized by polymorphous lymphoplasmacytic infiltrate
IgG4-related disease (IgG4-RD): Subgroup of idiopathic orbital inflammation (IOI) IEI lesions where multisystem manifestation is present with positive cell immunostain showing IgG4-rich plasma cells
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
IgG4-Related Disease Subtype Additional Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Bhatkar S et al: Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome. Neuroradiol J. 33(6):501-7, 2020
Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
Munawar K et al: Cavernous sinus lesions. Clin Imaging. 68:71-89, 2020
Rizzo E et al: Idiopathic hypertrophic pachymeningitis: does earlier treatment improve outcome? Children (Basel). 8(1), 2020
Zhao Y et al: Imaging features, clinicopathological analysis and diagnostic strategy of IgG4-related hypertrophic pachymeningitis. Ann Palliat Med. 9(5):2551-8, 2020
Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017
De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res.65(1):386-94, 2016
Dash GK et al: Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. J Neurol Sci. 350(1-2):51-60, 2015
Hung CH et al: Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis. Headache. 55(2):252-64, 2015
Bhatti RM et al: IgG4-related disease of the head and neck. Adv Anat Pathol. 20(1):10-6, 2013
Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
Tian CL et al: Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis. Chin Med J (Engl). 125(8):1513-6, 2012
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
Battineni ML et al: Idiopathic hypereosinophilic syndrome with skull base involvement. AJNR Am J Neuroradiol. 28(5):971-3, 2007
Mangiardi JR et al: Extraorbital skull base idiopathic pseudotumor. Laryngoscope. 117(4):589-94, 2007
Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
Lee EJ et al: MR imaging of orbital inflammatory pseudotumors with extraorbital extension. Korean J Radiol. 6(2):82-8, 2005
Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
Narla LD et al: Inflammatory pseudotumor. Radiographics. 23(3):719-29, 2003
Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
Cho YS et al: Inflammatory pseudotumour involving the skull base and cervical spine. J Laryngol Otol. 115(7):580-4, 2001
Bencherif B et al: Intracranial extension of an idiopathic orbital inflammatory pseudotumor. AJNR Am J Neuroradiol. 14(1):181-4, 1993
Nonspecific, nonneoplastic benign inflammatory lesion without identifiable local or systemic causes characterized by polymorphous lymphoplasmacytic infiltrate
IgG4-related disease (IgG4-RD): Subgroup of idiopathic orbital inflammation (IOI) IEI lesions where multisystem manifestation is present with positive cell immunostain showing IgG4-rich plasma cells
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
IgG4-Related Disease Subtype Additional Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Bhatkar S et al: Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome. Neuroradiol J. 33(6):501-7, 2020
Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
Munawar K et al: Cavernous sinus lesions. Clin Imaging. 68:71-89, 2020
Rizzo E et al: Idiopathic hypertrophic pachymeningitis: does earlier treatment improve outcome? Children (Basel). 8(1), 2020
Zhao Y et al: Imaging features, clinicopathological analysis and diagnostic strategy of IgG4-related hypertrophic pachymeningitis. Ann Palliat Med. 9(5):2551-8, 2020
Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017
De Virgilio A et al: Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res.65(1):386-94, 2016
Dash GK et al: Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. J Neurol Sci. 350(1-2):51-60, 2015
Hung CH et al: Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis. Headache. 55(2):252-64, 2015
Bhatti RM et al: IgG4-related disease of the head and neck. Adv Anat Pathol. 20(1):10-6, 2013
Fujita A et al: IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 32(7):1945-58, 2012
Tian CL et al: Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis. Chin Med J (Engl). 125(8):1513-6, 2012
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
Battineni ML et al: Idiopathic hypereosinophilic syndrome with skull base involvement. AJNR Am J Neuroradiol. 28(5):971-3, 2007
Mangiardi JR et al: Extraorbital skull base idiopathic pseudotumor. Laryngoscope. 117(4):589-94, 2007
Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
Lee DK et al: Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy. Otolaryngol Head Neck Surg. 135(1):144-8, 2006
Lee EJ et al: MR imaging of orbital inflammatory pseudotumors with extraorbital extension. Korean J Radiol. 6(2):82-8, 2005
Kupersmith MJ et al: Idiopathic hypertrophic pachymeningitis. Neurology. 62(5):686-94, 2004
Narla LD et al: Inflammatory pseudotumor. Radiographics. 23(3):719-29, 2003
Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
Cho YS et al: Inflammatory pseudotumour involving the skull base and cervical spine. J Laryngol Otol. 115(7):580-4, 2001
Bencherif B et al: Intracranial extension of an idiopathic orbital inflammatory pseudotumor. AJNR Am J Neuroradiol. 14(1):181-4, 1993
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