title: Management of orbital IgG4-related disease.
abstract: OBJECTIVE
  IgG4-related disease (IgG4-RD) is a systemic process that can cause significant orbital disease. It can affect both sexes and all ages, with irreversible consequences if left untreated. Diagnosis is currently based upon a combination of clinical and imaging evidence of tissue swelling or mass, serum evidence of elevated IgG4 levels and histopathologic evidence of inappropriate IgG4 presence. The cause of IgG4-RD is as of yet unclear; this lack of understanding and the dearth of prospective studies have limited our ability to manage patients effectively. In this review, we discuss the most recent published evidence regarding best-practice management of IgG4-related orbital disease.
  
  
  RESULTS
  Recent literature remains retrospective, and has focused on the use of corticosteroid therapy as a first-line treatment. Rituximab infusions have also received significant attention, among other second-line agents. Radiation therapy has been reported to be effective. Long-term monitoring for relapse, involvement of other organ systems and potential neoplastic transformation is required.
  
  
  CONCLUSIONS
  The management of orbital IgG4-RD will gain from more targeted therapy in the future as the underlying cause is better understood. In the meantime, randomized, controlled trials of varying treatment regimens would be of benefit.
authors.affiliation: Ophthalmic Plastic Surgery Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Lora R Dagi
authors.initials: LR
authors.lastname: Glass
authors.forename: Suzanne K
authors.initials: SK
authors.lastname: Freitag
mesh_terms: D001327:Autoimmune Diseases; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G; D009916:Orbital Diseases; D000069283:Rituximab
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D000069283:Rituximab