IgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two Cases

Cler, Samuel J.; Sharifai, Nima; Baker, Brandi; Dowling, Joshua L.; Pipkorn, Patrik; Yaeger, Lauren; Clifford, David B.; Dahiya, Sonika; Chicoine, Michael R.

doi:10.1016/j.wneu.2021.03.054

Cited by 12 publications

(16 citation statements)

References 105 publications

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“…The most recent guidelines from the American College of Rheumatologists and European League Against Rheumatism Guidelines (5) have shifted toward a point-based system where IgG4-RD diagnosis is based on a spectrum of classic features and absence of other plausible diagnoses. Skull base manifestations, including temporal bone disease were, however, underrepresented by these guidelines (13). Various studies have found that the exact histological findings vary greatly depending on the tissue affected and clinical presentations (13,14).…”

Section: Introductionmentioning

confidence: 99%

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

et al. 2022

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ObjectiveIgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone.Databases ReviewedOvid MEDLINE, EMBASE, Cochrane Library, and Google Scholar.MethodsWe used the following search keywords: “lgG4-RD,” “skull,” “skull base,” “cranial,” “temporal bone,” “inner ear.” We additionally manually searched the bibliographies of relevant articles. The JBI Critical Appraisal Checklist for Case Reports and Case Series was used to assess the risk of bias; because of the scarcity of the reports, data were available through limited case series and reports; thus, data synthesis was not possible.ResultsWe identified 17 studies with 22 cases with temporal bone involvement. The most common presenting symptoms were hearing loss, otalgia, and headache. The mastoid and petrous bone were the most affected anatomical areas. Both computed tomography and magnetic resonance imaging were used. Biopsies showed the characteristic lymphoplasmacytic infiltrate in all cases, with histopathology being the diagnostic modality that set the diagnosis. Most patients were treated with corticosteroids ± surgery or a combination of corticosteroids and immunosuppressants with 95.5% symptomatic response and disease control.ConclusionIgG4-RD of the temporal bone radiologically manifests as space-occupying, lytic lesions; clinically, it presents with vague otological symptoms. Diagnosis involves a thorough workup, with histopathology being crucial in setting a definite diagnosis. IgG4-RD tends to respond well to systemic corticosteroids, whereas surgery is mostly required for diagnostic purposes.

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Section: Introductionmentioning

confidence: 99%

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

et al. 2022

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“…Isolated IgG4-RD invasion of cavernous sinus is even more rare. [3,5,6,18,19,24,25,29,35] In this report, we have discussed about treatment given to a patient who was presented with cavernous sinus and orbit invasion IgG4-RD, and required surgical intervention to attain adequate functional improvement. So far, very few population-based studies of IgG4-related disease have been conducted.…”

Section: Discussionmentioning

confidence: 99%

“…Corticosteroids and immunosuppressive agents may exacerbate similar appearing lesions such as osteomyelitis or alter the diagnostic yield of a biopsy and therefore should not be started unless there is a suspected diagnosis of IgG4-RD. [ 5 - 25 , 29 , 32 - 37 ]…”

Section: Discussionmentioning

confidence: 99%

“…is is the main reason for the delay in diagnosis and proper management of the disease. [2][3][4][5][6]12,13,19] In this report, we will describe the unique case of a patient with IgG4-RD of cavernous sinus and orbit invasion, which was treated through surgical intervention after conservative management and steroid therapy failed to work.…”

Section: Introductionmentioning

confidence: 99%

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Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

Mayeku

Deisch

López-González

2021

Surgical Neurology International

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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“…While reports of skull-base lytic lesions in IgG4 patients are rare, reports of calvarial involvement are exceedingly rare. In a 2021 systematic review of more than 1,352 cases of IgG4-RD, 20 357 cases included head and neck involvement. Of these, only a handful of cases described calvarial involvement, mainly of the temporal bone (eight cases).…”

Section: Discussionmentioning

confidence: 99%

IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case

Suisa

Soustiel

Grober

2021

Journal of Neurosurgery: Case Lessons

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BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.

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IgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two Cases

Cited by 12 publications

References 105 publications

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

IgG4-Related Sclerosing Disease of the Temporal Bone: A Systematic Review

Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case

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