[IgG4-related disease with nasopharyngeal malignancy-like manifestations]

Abstract

Objective:The purpose of this article was to discuss the clinical features and imaging characteristics of IgG4-related disease（IgG4-RD） in order to identify nasopharyngeal IgG4-RD at an early stage. Methods:The basic information of the patients, including age, sex, symptoms, disease duration and treatment process, was collected through the electronic case system. Laboratory tests including nasal endoscopy, EBV levels, IgG4 levels and C-reactive protein levels were recorded during hospitalization and outpatient follow-up. All radiological imaging and postoperative pathology data are collected, analyzed and summarized. Results:All patients underwent partial excisional biopsy of the lesion. The pathological findings showed inflammatory granulomatous and fibrous tissue hyperplasia with a high infiltration of lymphocytes, plasma cells and neutrophils, and immunohistochemistry examination showed IgG4+ plasma cells were more than 10 per high magnification field. Combining medical history, imaging, serological findings and relevant treatment, all four patients were diagnosed with IgG4-associated disease. And their symptoms improved significantly after hormonal and immunosuppressive treatment. Conclusion: IgG4-RD has a highly similar clinical presentation with nasopharyngeal carcinoma. Differentiation from IgG4-RD should be considered for those pathology cannot be clarified by multiple biopsies. Timely diagnosis of IgG4-RD is important to prevent secondary organ damage in patients with active disease.

Keywords: IgG4-related disease; diagnose; nasopharyngeal neoplasms; rare diseases.