IgG4-Related Disease of Bilateral Temporal Bones

Li, Lilun; Ward, Bryan K.; Cocks, Margaret; Kheradmand, Amir; Francis, Howard W.

doi:10.1177/0003489416672476

Cited by 15 publications

(16 citation statements)

References 8 publications

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“…Similarly, since skull base IgG4‐RD can also present in the setting of systemic disease, clinical and radiological screening for the presence of systemic disease is prudent given the possibility of identifying more readily accessible biopsy sites elsewhere. Nevertheless, consideration of surgical excision of isolated masses that are not intimately involved with surrounding neurovasculature is reasonable, especially since evidence suggests that gross total resection may facilitate long‐term remission, particularly for patients that do not have concomitant systemic disease . The role of radiotherapy or radiosurgery to treat symptomatic skull base disease is unknown, and the current study's results were largely equivocal, with the one case treated with radiosurgery and subsequent medical therapy demonstrating symptomatic improvement while the case treated with fractionated radiotherapy showed no post‐radiotherapy improvement.…”

Section: Discussionmentioning

confidence: 73%

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

et al. 2019

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IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. Methods: Review of all cases at three tertiary-referral centers since disease description in 2003. Results: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. Conclusions: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.

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Section: Discussionmentioning

confidence: 73%

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

et al. 2019

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“…We identified 22 cases of IgG4-RD with temporal bone involvement from 17 different studies (Table 1) (18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35). Results are presented exactly as reported in original publications without solicitation of additional information from their authors.…”

Section: Resultsmentioning

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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“…Neuroimaging techniques are mandatory to defining the extent of the disease inside and outside the temporal bone. Bone erosion of the labyrinth was observed, as well as pachymeningeal enhancement of the posterior fossa [ 148 , 149 ].…”

Section: Audiovestibular Pathology Associated With Systemic Autoimmentioning

confidence: 99%

“…Surgical resection with adjunctive corticosteroids may be required for temporal bone IgG4-RD, which has been suggested to follow a more aggressive course. In all reported cases of unilateral temporal bone IgG4-RD, tumor size reduction and disease remission were achieved with a combination approach of mastoidectomy and medical management [ 148 ].…”

Section: Audiovestibular Pathology Associated With Systemic Autoimmentioning

confidence: 99%

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Girasoli

Cazzador

Padoan

et al. 2018

Journal of Immunology Research

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The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere's disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet's disease, Cogan's syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides.

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IgG4-Related Disease of Bilateral Temporal Bones

Cited by 15 publications

References 8 publications

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

Manifestations of Skull Base IgG4‐Related Disease: A Multi‐Institutional Study

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

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

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