Pathology Discovery

Pathology Discovery

ISSN 2052-7896
Review

Orbital IgG4-associated diseases

Martina Zavorkova1†, Josef Richter2†, Vaclav Vetvicka3†* and Ivana Liehnova1†

*Correspondence: Vaclav Vetvicka vaclav.vetvicka@louisville.edu

3. University of Louisville, Department of Pathology, Louisville, KY, USA.

Author Affiliations

†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.

Abstract

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

ISSN 2052-7896
Volume 5
Abstract Download