Three cases of IgG4-related orbital inflammation presented as unilateral pseudotumor and review of the literature.

Abstract

IgG4-related disease is an emerging disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. In IgG4-related disease, tumor-like lesions develop in multiple organs, such as the lacrimal gland, salivary gland, lung, pancreas, kidney, and lymph nodes. We report here three cases of IgG4-related orbital inflammation that presented as unilateral pseudotumors. The patients all were men, with an age range of 65-75 years. The patients had been pointed out unilateral intra-orbital masses, and histopathological examinations revealed marked accumulation of IgG4-positive plasma cells (IgG4/IgG ratio: 51.1-71.6%) with fibrosis. But storiform fibrosis was seen in only one case, and no obliterative phlebitis was seen. The serum levels of IgG4 were increased to 178-670 mg/dL. The masses had well-defined homogeneous signal intensities, and they were hypo-intense on T1-weighted MR images and iso-intense on T2-weighted MR images. Gadolinium enhanced mass lesions in two cases. All orbital mass lesions responded well to corticosteroid treatment.

• Authors: Tomoki Origuchi, Hiroki Yano, Hideki Nakamura, Akiyoshi Hirano, Atsushi Kawakami
• Journal: Rheumatology international (Rheumatol Int) Vol. 33 Issue 11 Pg. 2931-6 (Nov 2013) ISSN: 1437-160X [Electronic] Germany
• PMID: 23138265 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Immunoglobulin G

Topics

• Aged
• Autoimmune Diseases (immunology, pathology)
• Humans
• Immunoglobulin G (immunology)
• Male
• Orbital Diseases (immunology, pathology)
• Orbital Pseudotumor (immunology, pathology)
• Plasma Cells (pathology)