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Speaker provides updates on management of Behçet’s syndrome
DESTIN, Fla. – A speaker here at the Congress of Clinical Rheumatology provided an update on Behçet’s syndrome, including a review of its clinical manifestations and new therapy options.
“The goal was to review the syndrome, examine other disorders that could mimic it and provide treatment options that emphasize newer and evolving therapies,” Kenneth T. Calamia, MD, professor of medicine and former chief in the Division of Rheumatology at Mayo Clinic, said.
Although rare in the United States, Behçet’s syndrome affects many parts of the world, Calamia said. Its cause is unknown, but it typically involves vasculitis, ulcerations on the skin, mouth and genitalia, swelling of parts of the eye, as well as pain, swelling and stiffness of the joints.
More severe problems can include meningitis, blood clots, digestive inflammation and blindness.
Kenneth T. Calamia
“The importance of the diagnosis of Behçet’s syndrome is related to the presence of risk of uveitis, central nervous system (CNS) disease and vascular manifestations,” Calamia said. “Younger patients and males are at greater risk of complications.”
Behçet’s syndrome has distinguishing factors that separate it from classic autoinflammatory disorders. It normally occurs in unprovoked inflammatory episodes and is innate to immunity abnormalities, Calamia said. It is also polygenetic and its presentation of uveitis differs than that of similar disorders.
About one in four patients with Behçet’s syndrome will develop large vessel involvement, Calamia said, which could include systematic and pulmonary arterial vasculitis, venous occlusions and varices. He said this could hinder prognosis, make treatment options unclear and lead to high percentages of morbidity and mortality. However, there are suggested methods to use in the treatment of patients with Behçet’s syndrome.
“Treatment depends on the clinical expression and prognosis, determined by age, gender and disease duration,” Calamia said. “Morbidity can be determined by eye involvement, vascular and CNS disease, and mortality is primarily determined by vascular and CNS disease.”
Calamia said according to the European League Against Rheumatism (EULAR) recommendations, patients with inflammatory eye disease of the posterior segment should receive azathioprine (AZA) and systematic steroids. For parenchymal CNS disease, rheumatologists should consider corticosteroids, interferon-alpha (INF-α), AZA, cyclophosphamide, methotrexate and tumor necrosis factor-alpha. Cyclosporin A (CSA) should be avoided unless essential for intraocular inflammations.
He said previous research also suggests the use of T-cell modulation, alemtuzumab, bone marrow transplantation, autologous stem cell transplantation, use of anti-tumor necrosis factor agents in Behçet’s syndrome. INF-α could be considered in posterior or panuveitis Behçet’s syndrome and gevokizumab in Behçet’s uveitis, Calamia added.
He said the management of deep vein thrombosis (DVT) should include steroids, AZA, cyclophosphamide (CTX) or CSA, and there is no evidence of benefit from anticoagulants in the management of DVT. He said CTX and steroids are recommended for pulmonary and peripheral arterial aneurysms. – by Shawn M. Carter
Reference:
Calamia KT. Behçet’s syndrome: Update including new therapies. Presented at: Congress of Clinical Rheumatology; May 12-15, 2015; Destin, Fla.
Disclosure: Calamia reports he received research support from Celgene and that the presented data contained discussion of unapproved agents for the treatment of Behçet’s syndrome.