Ocular and Systemic Manifestations of Behçet's Disease: Exploring Pathogenesis and Therapeutic Modalities

Behçet’s disease (BD) is a rare, chronic, relapsing multi-system inflammatory disorder, first described by Hulusi Behçet in 1937, and classically characterized by ocular inflammation, recurrent oral ulcers & genital ulcers. It is presently identified as a variable-vessel vasculitis affecting arteries and veins irrespective of size,thereby contributing to widespread organ involvement. Bilateral occlusive retinal vasculitis and recurrent uveitis are the primary ocular manifestations associated with BD, representing the major sight-threatening complications that can lead to severe visual impairment or blindness if untreated. BD is more prevalent along the historic Silk Road, including Iran, Korea, Japan, and Turkey, despite being observed globally.The etiology remains unknown, but multifaceted interactions between genetic predisposition, especially HLA-B51, & environmental or infectious inducers lead to pathogenesis, facilitated largely by imbalanced Th1 and Th17 immune responses. Diagnosis is based on clinical criteria, as disrupted biomarker exists, with ocular involvement, oral and genital ulcers, cutaneous lesions, and a positive pathergy test being significant features. Therapeutic approach requires a combination of corticosteroids for acute flares and immuno-suppressive for chronic disease control. Azathioprine, cyclosporine, and methotrexate are commonly used conventional treatment, although cyclosporine is contraindicated in neuro-Behçet’s disease. Among biologic therapies, interferon-α remains effective in stimulating sustained remission, while TNF-α inhibitors have shown high efficacy in refractory systemic and ocular disease. Timely diagnosis, early immunosuppression, and cautious biologic use are necessary to minimize relapses, resist irreversible tissue damage, and induce long-term prognosis in BD, as discussed in this review article.