[IgG4-related disease presenting with involvement of the eyes, pituitary gland and nervous system: A case report]

To report a case of IgG4-related disease (IgG4-RD) involving the eyes, pituitary gland, dura mater and temporal region simultaneously. The patient was a 55-year-old middle-aged male with a protracted course of more than 13 years. The main clinical manifestations included swelling of the left eye accompanied by exophthalmos, which gradually worsened, and progressive visual deterioration. Imaging examinations revealed multiple pseudotumorous masses in the bilateral extraconal spaces (superior to the bilateral superior rectus muscles and inferior to the bilateral inferior rectus muscles) and the left optic nerve sheath area, pseudotumorous thickening of the bilateral temporal dura mater, sellar nodules, as well as pseudotumorous thickening of the muscular tissues in the left temporal and maxillofacial regions. Laboratory tests showed elevated serum IgG4 levels. Pathohistological biopsy of the ocular tissue demonstrated lymphoid hyperplasia accompanied by whorled or storiform proliferative fibrous tissue, formation of lymphoid follicles, and plasma cell infiltration. Immunohistochemical staining was positive for IgG4, with the ratio of IgG4-positive plasma cells to total IgG-positive plasma cells exceeding 70%. After treatment with hormones combined with immunosuppressants, the lesions in these areas shrank and some disappeared. Therefore, the comprehensive diagnosis was IgG4-RD.