LONDON – A EULAR task force issued the first update to recommendations for managing Behçet’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. Gülen 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 Behçet’s disease, said Dr. Hatemi, convenor of the task force and a rheumatologist at Istanbul University.
For mucocutaneous involvement, the update included five items that all received a “strong” recommendation from the task force: For an oral or genital ulcer, use a topical agent, such as a local steroid. 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. And 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 for managing eye involvement along with one conditional recommendation. 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 along with 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. A conditional recommendation said patients with refractory venous thrombosis could be considered for treatment with a monoclonal TNF-alpha antagonist, along with an anticoagulant if the patient’s risk for bleeding was generally low and a coexistent pulmonary artery aneurysm was ruled out.
Management of arterial aneurysms received the other two strong recommendations. The panel recommended high-dose glucocorticoid plus cyclophosphamide for a pulmonary artery aneurysm, followed by a monoclonal TNF-alpha antagonist for refractory cases. Patients with these aneurysms who are at high risk for major bleeding should undergo embolization in preference to open surgery. When patients have aortic or peripheral artery aneurysms, treatment should start with cyclophosphamide and a corticosteroid before an aneurysm repair is attempted. But surgery or stenting of the aneurysm should not be delayed when patients are symptomatic.
Gastrointestinal involvement received one strong and two conditional recommendations. The panel strongly recommended confirming gastrointestinal involvement using endoscopy, imaging, or both, while also ruling out treatment with a nonsteroidal anti-inflammatory drug, inflammatory bowel disease, or an infection such as tuberculosis as the cause of gastrointestinal symptoms. One of the conditional recommendations called for an urgent surgical consult when patients have perforation, major bleeding, or obstruction. The second conditional recommendation called for considering glucocorticoid treatment to treat an acute exacerbation of gastrointestinal involvement. Additional treatment options to pair with a glucocorticoid include a disease-modifying drug such as 5-aminosalicylic acid or azathioprine. For patients with severe or refractory gastrointestinal symptoms or both, a monoclonal TNF-alpha antagonist or thalidomide is another potential option.
The panel issued two strong recommendations for managing nervous system involvement. The top treatment option for parenchymal involvement is a high-dose glucocorticoid followed by slow tapering while also treating with an immunosuppressant such as azathioprine. Treatment with cyclosporine should be avoided, the panel said. Treatment with a monoclonal TNF-alpha antagonist is an option to consider as first-line treatment for patients with severe nervous system involvement or for those with refractory disease. The second strong recommendation was to treat a cerebral venous thrombus with a high-dose glucocorticoid followed by tapering, with short-term anticoagulant treatment as an option. Patients also need screening for the presence of vascular disease at an extracranial location.