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

Abstract

The discovery of systemic disease related to raised tissue and serum immunoglobulin G4 (IgG4) is changing diagnostic and therapeutic practice in many medical specialties.

Rituximab in IgG4-related inflammatory disease of the orbit and ocular adnexae

“…2,3 Since the submission of a review on adnexal IgG4-related disease, 4 papers have been published on the use of Rituximab for systemic IgG4-related disease and data are now emerging on the use of Rituximab for IgG4-specific orbital disease.…”

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.…”

Orbital IgG4-associated diseases

“…These can include changes in immunological and biochemical parameters. However, further development of new diagnostic steps allowing precise diagnosis is needed [38]. In addition, orbital soft tissue and periorbital structures with intact lacrimal glands with clinical signs of proptosis can be affected.…”

“…IgG4-RD can influence any cellular system in different organs. Eye problems are one of the main manifestations of this disease [12,17,38]. Table 1 summarizes the guidelines for diagnosis of IgG-RD, Table 2 shows our current knowledge of clinical manifestations, occurring in different frequencies and often in connection with -RD (1,7,8,9,12,13,14,15, 16,18,22,34,38,40).…”