Treatment

Treatment depends on organ involvement and severity of disease (Table 3). Mild orogenital ulcerative disease can be treated on a periodic basis; however, severe systemic disease may require chronic immunosuppressive therapy. For mild oral or genital ulcerations, topical anesthetics or corticosteroids can be used. Oral colchicine (0.6-1.8 mg/day) and dapsone are also effective. For more severe mucocutaneous disease, oral corticosteroids, azathioprine, or methotrexate can be used. Thalidomide has also been shown to be effective for more severe mucocutaneous disease, but due to its association with birth defects, its use in the United States requires adherence to the federally mandated drug use pathway (32). Interferon-a (IFN-a) therapy has also been used for severe mucocutaneous and systemic manifestations of disease (33). Additionally, antitumor necrosis factor-a (INF-a) therapy with etanercept or infliximab has been shown to improve mucocutaneous lesions and features of systemic disease (34).

For ocular disease, past treatment algorithms included the use of high-dose corticosteroids, cyclosporine, and cytotoxics such as chlorambucil and cyclophosphamide. However, there is growing evidence that anti-TNF-a therapy can lead to rapid resolution of uveitis and retinal vasculitis, and its early use in severe ocular disease should be considered (34,35). The anti-TNF-a agent best studied for ocular Adamantiades-Behget's is infliximab. Although some have seen improvement in disease with a single infusion, to maintain disease control, infliximab is usually dosed at 5 mg/kg at four- to eight-week intervals once drug loading at weeks 0, 2, and 6 has been performed (35,36). The duration of therapy needed for control of eye disease is unknown, but many propose that immunosuppressive therapy be continued for at least one year after resolution of inflammation, although many patients may require prolonged therapy to control disease, as well as concomitant use of other immunosuppressive agents to ensure control.

For nervous system disease, treatment with potent immunosuppressive agents, including use of high-dose corticosteroids and antimetabolite or cytotoxic therapies, is needed. The use of anti-TNF-a agents in nervous system manifestation of disease is not well studied, but may also be beneficial (37).

For superficial thrombophlebitis, local therapy including heat and cold along with aspirin or other nonsteroidal anti-inflammatory therapy may be adequate. If deep venous thrombosis is present, full anticoagulation should be considered.

Determining who to screen for pulmonary aneurysms is difficult. Some advocate evaluating all patients with Adamantiades-Behget's for pulmonary aneurysms, but this is costly and may not be necessary for all patients. Certainly, if a patient presents with hemoptysis, further imaging should be performed, with contrasted CT imaging being the preferred initial diagnostic test. If pulmonary embolism is suspected, evaluation for the presence of aneurysms should also be performed, as there have been cases of death reported in patients who are anticoagulated for presumed embolus, with resultant severe pulmonary hemorrhage from undiagnosed aneurysms (16,25). If pulmonary artery aneurysms are identified, combinations of cytotoxic therapy and high-dose corticosteroids, followed by long-term immunosuppressive therapy, are recommended, although no controlled trials have been performed (16). The role of anti-TNF-a therapy in the treatment of arterial aneurysms is unknown. For patients with active bleeding from an aneurysm, methylpredni-solone dosed at 1000 mg intravenously daily for three to five days, along with pulse-dose cyclophosphamide, should be administered, with consideration of embolization or surgical intervention if symptoms are not controlled (16).

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