The disease course in Adamantiades-Behçet's is characterized by exacerbations and remissions, with a decrease in severity as patients become older (38). Patients should be counseled that while in some cases, the disease remits after a few years, in others, longer-term medications are required to control symptoms. The most severe disease is seen in younger males of Mediterranean descent, but even in this group, disease tends to be less damaging as patients age (38). Oral or genital ulcerations are usually the initial findings of disease, with other manifestations including ocular and nervous system involvement occurring simultaneously, although in some cases, eye and neurologic disease may occur years after ulcerations first appear. In addition, chronic uveal inflammation can lead to significant vision loss or blindness in over 50% of patients with untreated eye disease (39) and requires close ophthalmologic follow-up to ensure that inflammation is controlled.
Mortality occurs usually from rupture of pulmonary artery aneurysms, large-vessel thrombosis, severe GI involvement with perforations or the development of the Budd-Chiari syndrome, or infections as a result of immunosuppressive therapy. Mortality is highest in young male patients of Turkish descent, with standardized mortality rates in this group up to 10 times greater than normal, with decreases in these ratios as age at diagnosis increases and as duration of follow-up continues (39). Mortality rates from Adamantiades-Behget's are lower for patients of European descent, although exact rates are difficult to determine, due to the rarity of disease.
IFN-a2a has been used in a number of trials, and some data regarding remission rates in Adamantiades-Behget's are available. In one study of patients with panuveitis, IFN-a2a was used for a mean duration of 40 months with remission in 9/10 subjects (40). There is little data regarding the long-term remissions of other manifestations of disease with IFN-a2a, however. A review published in 2004 showed that the use of IFN-a2a resulted in improvement of articular disease and mucocutaneous disease in 95% and 86% of treated patients, respectively (41), but long-term data regarding efficacy with neurologic and vascular disease and overall mortality need to be obtained. The more recent use of anti-TNF agents, specifically infliximab, in Adamantiades-Behget's will likely alter the morbidity and mortality seen in the past with this disease, but further studies are needed.
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