Globally, males are affected more frequently than females.In an epidemiologic study, 56 percent of patients with Behçets disease developed ocular involvement at a mean age of 30. Ocular involvement was the first manifestation of Behçets disease in 8.6 percent of patients. Ocular Behçets disease with involvement of the optic nerve is rarely reported. Among patients with ocular Behçets disease funduscopic findings of optic atrophy, and optic disc paleness have been identified with a frequency of 17.9 percent and 7.4 percent, respectively. Other fundoscopic findings include vascular sheathing (23.7%), retinal hemorrhage (9%), macular edema (11.3%), branch retinal vein occlusion (5.8%), and retinal edema (6.6%). However, optic atrophy was the most significant cause of visual impairment identified in 54 percent of patients with ocular Behçets disease and permanent visual impairment.The prevalence of this disease increases from North to South. It follows a more severe course in patients with an early age of onset particularly in patients with eye and gastrointestinal involvement. Pregnancy
With Behçets disease as a pre-existing disease in pregnancy or acquired, the pregnancy does not have an adverse effect on the course of Behçets disease and may possibly ameliorate its course. Still, there is a substantial variability in clinical course between patients and even for different pregnancies in the same patient.
However, B51 tends not to be found in disease when a certain SUMO4 gene variant is involved, and symptoms appear to be milder when HLA-B27 is present. At the current time, a similar infectious origin has not yet been confirmed that leads to Behçets disease, but certain strains of S. sanguinis has been found to have a homologous antigenicity.Vasculitis resulting in occlusion of the vessels supplying the optic nerve may be the cause of acute optic neuropathy and progressive optic atrophy in Behçets disease. Histological evaluation in a reported case of acute optic neuropathy demonstrated substitution of the axonal portion of the optic nerve with fibrous astrocytes without retinal changes. CNS involvement in Behçets disease may lead to intracranial hypertension most commonly due to dural venous sinus thrombosis and subsequent secondary optic atrophy. Diagnosis
There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy test. Behçets disease has a high degree of resemblance to diseases that cause mucocutaneous lesions such as Herpes simplex labialis, and therefore clinical suspicion should be maintained until all the common causes of oral lesions are ruled out from the differential diagnosis.Visual acuity, or color vision loss with concurrent mucocutaneous lesions or systemic Behçets disease symptoms should raise suspicion of optic nerve involvement in Behçets disease and prompt a work-up for Behçets disease if not previously diagnosed in addition to an ocular work-up.