Intravenous immunoglobulin in eye involvement.

Ocular inflammation may affect all eye layers: conjunctiva, sclera, uvea, and orbital tissues. The main eye involvement requiring a systemic treatment is uveitis, which represents a heterogeneous group of rare diseases, most of which are sight-threatening. In around 40% of uveitis cases an underlying systemic disease, often of autoimmune origin, can be identified. In autoimmune diseases with intraocular inflammation (IOI), uveitis may be the first clinical manifestation and may represent the most severe sign. Studies in animal models, especially in experimental autoimmune uveitis (EAU), offer the opportunity to investigate the pathogenicity of these disorders. The conventional treatment of IOI includes corticosteroids and immunosuppressive agents, which are efficient in around one-half of the patients; however, their effectiveness is also limited by their iatrogenicity. The effects of intravenous immunoglobulin (IVIg) on ocular inflammation have been investigated in a wide spectrum of autoimmune/systemic diseases. Most publications are case series or open trials. They show favorable results in a subset of indications including mainly ocular cicatricial pemphigoid, Vogt-Koyanagi-Harada syndrome, or birdshot disease. Efficacy results are more debated in other conditions, such as inflammatory demyelinating optic neuritis. In other diseases with IOI (Wegener disease, Behcet's disease, inflammatory myositis), only case reports are available, suggesting that IVIg may be of some interest. These observations support the need for controlled trials to demonstrate the efficacy of IVIg and assess their potential steroid-sparing effect.