Intraocular and orbital IgG4-related sclerosing disease

1. Hideki Tsuji¹ ²
2. Takashi Fujishiro² ³
3. Takashi Shigeeda²
4. Tatsuya Mimurai^{2 4}
5. Yutaka Takazawa⁵
6. Takayoshi Kojima^{2 3}

¹Department of Ophthalmology, The Cancer Institute Hospital , Tokyo, Japan
²Department of Ophthalmology, University of Tokyo Graduate School of Medicine, Tokyo, Japan
³Department of Ophthalmology, Saitama Red-Cross Hospital, Saitama, Japan
⁴Department of Ophthalmology, Toranomonn Hospital , Tokyo, Japan
⁵Department of Pathology, University of Tokyo Graduate School of Medicine, Tokyo, Japan

Purpose : To report a case of intraocular and orbital IgG4-related sclerosing disease.

Methods : Case report.

Result: A 57-year-old man presented progressive painful intraocular tumor in the left eye. Magnetic Resonance Imaging revealed intraocular and orbital tumor with high signal in T1 and low in T2. The patient was suffered severe pain with decreased the best-corrected visual acuity to Hand Motion, he underwent tumor resection with enucleation. The serology showed raised IgG4 levels and histopaphologic examination revealed abundant IgG4-positive plasma cells and dense fibrosis. The diagnosis was IgG4-related sclerosing disease.

Conclusion: IgG4-related sclerosing disease can invade ocular and orbit. To the best of our knowledge, this case is the first documentation of intraocular invasion of IgG4-related sclerosing disease.