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

Abstract

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.

IgG4-Related Disease of Temporal Bone Presenting as Unilateral Mastoiditis and Cerebral Thrombosis in a 22-Year-Old Man

“…The most frequently reported symptoms are vague, such as hearing loss, otalgia, and headache [9], similar to the current case. Symptoms of asthma or allergies are present in approximately 40% of patients whereas vascular involvement has 15-25% rates and the aorta is the vessel most commonly affected [12].…”

“…Clinically, it presents with vague otological symptoms. The most common are progressive hearing loss, otalgia, and headache [9]. Ear fullness, pressure, and otorrhea are other possible symptoms [10].…”

IgG4-related inflammatory pseudotumor involving the temporal bone disguised as meningioma: a case description and literature analysis

“…In the present case, seizures were the only clinical symptom. In this case, it was the temporal bone and meninges that were involved, which was different from the most common involvement of the mastoid process in existing reports ( 6 ). The lesion was observed to be completely extradural.…”

“…Meningeal IgG4-RD symptoms typically occur in isolation with no other organ involvement when serum IgG4 concentrations are normal or barely elevated ( 33 ). The most frequent symptom of IgG4-RD is hearing loss, while seizures are rare ( 6 , 9 ). In the present case, seizures were the only clinical symptom.…”

“…Nearly 20% of patients with IgG4-RD often have normal concentrations of serum IgG4 (5). A wide range of IgG4-RD lesions has been found to affect the temporal bone, including space-occupying lesions that cause cranial nerve palsies and locally invasive lesions that cause bony destruction (6). IgG4-RD lesions must also be differentiated from benign and malignant tumors involving the temporal bone, such as hemangioma, meningioma, cancer, and lymphoma.…”