Abstract
A 77-year-old man with a painless and bilateral submandibular swelling was referred to our hospital. He had already been treated with systemic prednisolone for retrobulbar neuritis. Laboratory examinations showed high serum IgG4 concentrations, and a biopsy of the submandibular gland revealed the infiltration of IgG4-positive plasmacytes. Abdominal CT demonstrated tumor formation in the tail of the pancreas. These findings suggested the patient to have Mikulicz's disease associated with localized autoimmune pancreatitis. Treatment with prednisolone improved the swelling of the submandibular gland. As a result, when a paticent is suspected to have Mikulicz's disease, it is important to consider the possibility of systemic IgG4-related plasmacytic disease.
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