Orbital Inflammation With IgG4-Positive Plasma Cells

Abstract

To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens.

Systemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing ‘idiopathic’ from the nomenclature?

“…[22][23][24][25][26][27][28] IgG4-associated disease is now recognised as a systemic disease process. 22 Table 2 describes conditions once viewed as unique but which are now recognised by the IgG4-related systemic disease spectrum. 29 The role of immunoglobulin G4 in IOI As the name implies, the pathogenesis of IOI has remained elusive.…”

Rituximab for the treatment of IgG4-related orbital disease: experience from five cases

“…12,17,18 To the best of the authors' knowledge, only 13 cases of biopsyconfirmed IgG4-ROD treated with rituximab have been reported in the literature. [18][19][20][21]27 Over half of these cases did not specify the dosing regimen. In three of these cases, the treatment regimen was two doses of rituximab (1000 mg) 15 days apart, and with each dose, methylprednisolone (100 mg), diphenhydramine (25 mg), and acetaminophen (650 mg) were also given.…”

“…12,17 However, the literature regarding rituximab treatment for biopsy-proven IgG4-ROD is limited to a few case reports. [18][19][20][21]27 The follow-up in these reports was generally short, often less than 10 months, and therefore the long-term efficacy of rituximab in IgG4-ROD is not known.…”

Orbital inflammatory disease management

“…The disease can affect multiple tissues in the orbit, including the lacrimal gland, extraocular muscles, and soft tissue [21][22][23][24]. IgG4-RD is a relatively new entity, and although controversial, it may account or a sizeable proportion of what was previously thought to be idiopathic orbital inflammation.…”