Behcet's update includes guidance on mucocutaneous manifestations.
LONDON -- A EULAR task force issued the first update to recommendations for managing Behcet's disease since 2008, with revised recommendations that reflect expanded use of biologic agents, and increased evidence to guide management of gastrointestinal involvement, use of anticoagulants in patients with venous involvement, and use of surgical and interventional treatments, Dr. Gulen Hatemi said while presenting the update at the European Congress of Rheumatology.
The task force, which included more than 20 members, identified 304 articles to apply to the update, and produced five overarching principal and 18 specific recommendations divided among six categories of clinical manifestations of Behcet's disease, said Dr. Hatemi, convenor of the task force and a rheumatologist at Istanbul University.
For mucocutaneous involvement, the update included five "strong" recommendations from the task force: For an oral or genital ulcer, a topical agent, such as a local steroid, is recommended. Try colchicine first to prevent recurrent mucocutaneous lesions, especially when the dominant lesion is erythema nodosum or a genital ulcer. Treat papulopustular or acnelike lesions with topical or systemic agents, as when treating acne vulgaris. Coordinate treatment of leg ulcers, which can be caused by venous stasis or obliterative vasculitis, with a dermatologist and vascular surgeon. Azathioprine, thalidomide, interferon-alpha, a tumor necrosis factor (TNF)-alpha antagonist, or apremilast (Otezla) may be necessary for selected patients.
The task force issued two strong recommendations and one conditional recommendation for managing eye involvement. The first strong recommendation was that managing uveitis requires close collaboration with an ophthalmologist, with the goal of inducing and maintaining remission. Patients with an inflammatory eye disease affecting the posterior segment should receive treatment with azathioprine, cyclosporine, interferon-alpha, or a monoclonal TNF-alpha antagonist. Treatment with a systemic corticosteroid should occur only when combined with azathioprine or another systemic immunosuppressant.
The second strong recommendation was that patients who present with an initial or recurrent acute episode of sight-threatening uveitis should receive treatment with a high-dose glucocorticoid, infliximab, or interferon-alpha. Intravitreal injection with a glucocorticoid as an adjunct to systemic therapy is an option for patients with a unilateral exacerbation. The conditional recommendation was for patients with isolated anterior uveitis. When these patients have markers of a poor prognosis such as young age, male sex, or early disease onset--systemic treatment with an immunosuppressant is a possible option.
The panel issued three strong recommendations and one conditional recommendation for managing vascular involvement. One of the strong recommendations called for treating acute deep vein thrombosis with a glucocorticoid as well as an immunosuppresant such as azathioprine, cyclophosphamide, or cyclosporine.
The conditional recommendation said patients with refractory venous thrombosis could be considered for treatment with a monoclonal TNF-alpha antagonist--with an anticoagulant if the patient's risk for bleeding was generally low and pulmonary artery aneurysm was ruled out.
Dr. Hatemi has received research support from, received honoraria from, or has been a speaker for Abb Vie, Celgene, Merck Sharp & Dohme, and Pfizer.
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