IgG4-related Ocular Adnexal Disease Mimicking Thyroid-associated Orbitopathy

A 72-year-old man presented with bilateral eyelid swelling and redness. An orbital CT scan showed bilateral proptosis, extraocular muscle enlargement and swollen lacrimal glands, mimicking thyroid-associated orbitopathy (TAO) with Hashimoto's thyroiditis (HT). During the patient's clinical course, spontaneous remission of lung consolidation (35 × 26 mm) was seen. A diagnosis of IgG4-related disease (IgG4-RD) was made based on an elevated serum IgG4 level (1,020 mg/dL; normal, 4-108), predominance of IgG4-positive plasma cells (IgG4/IgG: 35/70 in HPF) in the lacrimal glands and typical features of Mikulicz's disease. This report provides a novel description of this unusual disease entity among HT, TAO and IgG4-RD.