• Rachel B Cain, Thomas V Colby, Vijayan Balan, Naresh P Patel, and Devyani Lal.
• Department of Otolaryngology, Mayo Clinic, Phoenix, Arizona, USA.
• Otolaryngol Head Neck Surg. 2014 Sep 1;151(3):496-502.

ObjectiveIgG4-related disease (IgG4RD) causing sinonasal and skull base pathology is uncommonly described. We present a series of suspected IgG4RD patients, with a pertinent review of the literature to highlight diagnostic challenges.Study DesignCase series.SettingAcademic tertiary care center.Subjects And MethodsCase series of patients with IgG4RD or suspected IgG4RD involving the sinonasal cavity and skull base.ResultsWe present 4 patients with atypical sinonasal and/or skull base disease who were noted to have IgG4-positive plasma cell infiltration on immunohistochemistry of biopsy specimens. IgG4RD, a recently described entity affecting multiple organs, is characterized by lymphoplasmacytic infiltration and often elevated serum IgG4. IgG4RD can masquerade as malignancy or infection but responds to glucocorticosteroid and immunosuppressant therapy. IgG4RD has been infrequently reported presenting as sinonasal or skull base lesions, and definitive diagnostic criteria for these regions are not established. In our series, IgG4RD was suspected in all 4 patients, but only 1 met all current criteria for definitive diagnosis. All 4 patients, however, responded to corticosteroid therapy, and 1 was placed on long-term azathioprine.ConclusionIgG4RD is rarely described in the sinonasal cavity and skull base, and specific diagnostic criteria for such disease have not been defined. We present a series of patients with IgG4-positive plasma cell inflammatory pathology who were suspected to have IgG4RD. Our series highlights diagnostic challenges associated with these patients. Tumefactive and destructive sinonasal-skull base lesions with a plasma cell-rich infiltrate should incite suspicion of IgG4RD, and immunohistochemistry for IgG4-positive plasma cells should be performed.© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

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