†These authors contributed equally to this work.
1. Ocni klinika UJEP Masarykovy nemocnice, Krajska zdravotni, a.s., Usti nad Labem, Czech Republic, USA.
2. Institute of Health, located in Usti nad Labem, Usti nad Labem, Czech Republic, USA.
IgG4-related disease (IgG4-RD) is a distinct entity that frequently occurs in an ophthalmic location. IgG4- RD is not limited to the orbit, but may also involve other anatomical structures in and around the eye. Careful clinicoradiologic examination and the use of immunohistological examination are key diagnostic methods. Serum IgG4 levels are neither sensitive nor specific enough for the diagnosis of IgG4-RD and should not be relied upon solely. Careful evaluation of histologic and immunophenotypic features and clinical correlation are required to distinguish orbital IgG4-RD from other inflammatory lesions in the orbit. Glucocorticoids are the primary therapeutic choice for treatment of IgG4-RD. Azathioprine or Mofetil can be used as a second possibility. Rituximab can be effective in the patients with relapse IgG4-RD.
Keywords: IgG4, eye, inflammation, ophthalmology