Behçet's disease is a multisystem inflammatory disease that has a chronic, recurrent course and particularly ophthalmological, oral, cutaneous, genital, vascular and neurological involvement. In the literature, the frequency of ocular involvement in childhood Behçet's disease varies between 27.3% and 80%. Eye involvement, which can cause blindness, is a serious complication of Behçet's disease. While recurrent anterior uveitis, occlusive retinal vasculitis, vitreous inflammation and hemorrhage, macular edema, infiltrates and soft exudates that may be accompanied by hypopyon are common ocular findings, retinal neovascularization, tears and macular ischemia are less common. A decrease in vision may also occur due to the side effects of the drugs used in the treatment. More than 3 ocular inflammatory attacks in a year suggest a poor visual prognosis. Early diagnosis and treatment are important to reduce the risk of vision loss in the long term.
The most important immunosuppressive agent in the treatment of childhood uveitis is corticosteroids, which can be applied topically, periocularly or systemically. Systemic and topical steroids provide rapid control of acute inflammation. Due to the undesirable effects of systemic corticosteroids on the immature immune, metabolic and skeletal system, as well as causing cataract formation and glaucoma, drugs such as methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, cyclophosphamide and anti-TNF agents are used to reduce long-term high-dose steroid side effects in the treatment of resistant uveitis. Other alternative immunosuppressive drugs can be used in the treatment of childhood uveitis. The development of neoplasia, myelosuppression, nephrotoxicity or hypertension may occur in the long term with these immunosuppressive agents. The patient should be informed about the potential side effects of the drugs and should be closely monitored. If undesirable effects occur, the drug dose should be reduced or a different drug should be used. Surgery may be required in cases of chronic recurrent inflammation, structural damage despite optimal treatment, cataracts, vitreous clouding and treatment-resistant glaucoma. Before cataract surgery, for complete control of inflammation, pre- and post-surgical steroid treatment, intraocular pressure control and immunosuppressives currently in use must be continued.
Behçet's uveitis can progress with acute fulminant attacks and cause severe vision loss. In these cases with a fulminant course, very aggressive systemic immunosuppressive treatment may sometimes be required to control the attacks.
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