Childhood Uveitis

Uveitis embraces a group of severe and disabling inflammatory diseases affecting the vascular layer of the eye (uvea). The International Uveitis Study Group developed a classification system based on the anatomical extension of ocular inflammation in anterior, posterior, and panuveitis. The clinical course of the disease may be acute, chronic, or recurrent. Among the possible etiologies, juvenile idiopathic arthritis stands for the most common cause in childhood, whereas cases without an identifiable origin, representing nearly half of total patients, are addressed as idiopathic. The diagnosis of uveitis requires close collaboration between pediatric rheumatologist and pediatric ophthalmologist in order to define the proper diagnostic work-up and therapeutic pathway. Compared to adults, childhood uveitis is characterized by poor prognosis and higher risk of secondary complications, and represents the third leading cause of blindness in developed countries. Targeted antimicrobial treatment is necessary for infectious uveitis, whereas for noninfectious chronic uveitis a step-by-step approach, in order to control local inflammation, achieve a corticosteroid-sparing effect, and reduce the risk of visual complications is currently recommended. In corticosteroid-resistant and corticosteroid-dependent cases systemic immunomodulatory agents should be considered, encompassing both immunosuppressive agents (methotrexate (MTX), azathioprine, mycophenolate mofetil) and biologic response modifier. MTX is considered the first-choice therapy in this setting. For patients intolerant or nonrespondant to MTX, antitumor necrosis factor alpha therapies (infliximab, adalimumab) represent an efficacious alternative treatment. Overall, the limited experience and the lack of randomized controlled trials hamper the possibility to draw general conclusion for treatment guidelines.