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IgG4 related disease (IgG4-RD) is a distinct entity that frequently occures in an ophthalmic location. IgG4 – RD is not limited to the orbit but may also involve other anatomical structures in and around the eye. A high level of suspicion for the diagnosis can be derived from careful clinico-radiologic examination, the use of immunohistological examination in the context of characteristic histopat...