IgG4 相關性疾病以眼框外腫來表現

IgG4-related disease presenting as peri-orbital swelling

57-year-old man presented with left peri-orbital swelling that developed over more than a year (Fig. 1a). He denied of having diplopia, restriction of ocular movements or visual field defects. The Schirmer’s test result was 4 mm OD and 1 mm OS (5 mins). The visual acuities were 0.9 OD and 0.5 OS. Laboratory studies showed normal values for free thyroxine, free triiodothyronine, thyroidstimulating hormone, antineutrophil cytoplasmic antibody, and negative results for autoantibodies related to collagen vascular diseases and Graves’ ophthalmopathy (anti-SS-A, anti-SS-B, antinuclear, anti-double stranded DNA, antithyroglobulin, and thyrotropin-receptor).The erythrocyte sedimentation rate, white cell count and C-reactive protein levels were within the normal range. The patient had elevated levels of serum IgG concentration as 1907 mg/dl (650–1,600 mg/dl), and serum immunoglobulin G4 (IgG4) as 1440 mg/dl (3–200 mg/dl).