Human immunoglobulins in intraocular inflammation.

Chronic intraocular inflammation (IOI) is a heterogeneous group of rare diseases, which represents one of the leading causes of acquired and treatable blindness in adults. The main anatomical site of inflammation is the uveal tract, which is the vascular organ of the eye, but uveitis is now used to describe IOI more globally. In around 40% of the cases of uveitis an underlying systemic disease, often of autoimmune origin, can be identified. In autoimmune diseases with 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, but their effectiveness is also limited by their iatrogenicity. The effects of intravenous immunoglobulins (IVIGs) on ocular inflammation have been investigated in a wide spectrum of autoimmune/systemic diseases. Most of the publications are case series or open trials. They show favorable results in a subset of indications, including mainly ocular cicatricial pemphigoid (OCP), Vogt-Koyanagi-Harada (VKH) syndrome, or birdshot disease. Efficacy results are more debated in other conditions such as inflammatory demyelinating optic neuritis. In other diseases with IOI only case reports are available, suggesting that IVIGs may be of some interest. These observations support the need for controlled trials to demonstrate the efficacy of IVIGs and assess their potential steroid-sparing effect.