LAUSR

Immunoglobulin G4 (IgG4) has been associated with many diseases in the ophthalmic field and other organ systems. Orbital pseudotumor and idiopathic orbital inflammation are two of these diseases. The presentation of these conditions may vary depending on the involvement of the eye or periorbital structures, including the uveitis. This inflammatory process may involve other organ systems outside the eye and periorbital region, including the central nervous system, salivary glands, lymph nodes, pancreas, kidneys, lungs, aorta, breast, thyroid, prostate, skin, and liver. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is characterized by the acute onset of symmetrical synovitis involving the upper and/or lower extremities with remarkable pitting edema in the soft tissue in the dorsa of the hands, giving the hands the appearance of “boxing gloves.” It has also been described as a unique form of synovitis and is mostly found in elderly men. The serum examinations in these patients have found elevated acute phase reactants and no rheumatoid factor (RF). An association between RS3PE and malignancies, such as malignant lymphoma, has also been reported. To the best of our knowledge, an association between IgG4 and RS3PE syndrome has never been reported.We report a case of IgG4associated uveitis complicated by RS3PE syndrome. A 62-year-old man with both ocular pain and blurred vision for a duration of 1 day was referred to our department. He had been treated for autoimmune pancreatitis 10 years ago and for mucosa-associated lymphoid tissue (MALT) lymphoma of the left orbit 1 year ago; he was treated using radiotherapy and chemotherapy and remained in complete remission (Figure 1A,B). He had been diagnosed with RS3PE syndrome by RF seronegative and symmetrical synovitis with pitting edema (Figure 1C) by a physician 6 months previously. At the first visit, the best-corrected visual acuities (BCVAs) were 16/20 in the right eye and 20/20 in the left eye. Intraocular pressures (IOP) were 23.3 mmHg in the right eye and 19.0 mmHg in the left eye. Both eyes had scleritis, fine keratic precipitates, and inflammatory cells in the anterior segments (Figure 2A–F), but there was no inflammation in the posterior segments. Serum examination detected an elevated C-reactive protein level (7.69 mg/dL), erythrocyte sedimentation rate (39 mm at 1 h), and immunoglobulin G4 level (721 mg/dL). The serum matrix metallopreteinase-3, anti-nuclear antibody, and RF levels were within normal limits. We diagnosed the patient with bilateral IgG4-related anterior uveitis with RS3PE syndrome and prescribed a typical steroid eye drop (0.1% betamethasone sodium phosphate, four times/day) and an oral steroid medication (prednisolone, 20 mg/day); the scleritis, bilateral anterior uveitis (Figure 2G,H), and symmetrical synovitis resolved immediately. At 1 week later, the visual acuities had improved to 20/20 in both eyes, and IOPs had decreased to the normal range. The oral steroid medication was tapered to 10 mg/day without any recurrences. Our patient had been diagnosed with autoimmune pancreatitis, MALT lymphoma in the left eye, and anterior uveitis. These diseases have been reported in relation to IgG4. Some malignancies, including malignant lymphoma, have been reported in association with the RS3PE syndrome, and these paraneoplastic RS3PE syndromes have been resistant to steroid treatment. In this case, the patient had MALT lymphoma in the left orbit 1 year ago, which remained in complete remission after systemic chemotherapy. Oral steroid medication immediately resolved the hand swelling. Therefore, it is possible that RS3PE syndrome is not a paraneoplastic