A solely ear-involved IgG4-related sclerosing disease with two-years following-up

Abstract Introduction Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a chronic fibro-inflammatory disease involving systemic multi-organ lesions, such as the salivary and lacrimal glands, lymph nodes and pancreas. The diagnosis of this disease is reliant upon clinical manifestations, laboratory tests, histopathologic results and radiological data. Some studies have found that IgG4-RSD has otological manifestations, whereas there were few studies introducing the diagnosis, therapy and long-term follow-up results of solely otological IgG4-RSD. Case summary Here, we report a case of IgG4-RSD involving the ear alone. A female presenting with otalgia and hearing loss underwent surgery, without hormone therapy. The pathological diagnosis was otological IgG4-RSD and no clinical or radiological signs of recurrence were observed at seven and twenty-four months follow-up. Discussion This case indicates that IgG4-RSD can invade the ear only, and that surgical therapy without hormone therapy is efficient for solely IgG4-RSD. Pathological results are crucial for diagnosis.