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Behçet Syndrome pp 279–305Cite as

Disease Management

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Abstract

The aim of treatment is suppression of symptoms and prevention of organ damage. Immunosuppressives constitute the mainstay of treatment. Male sex, young age, and short disease duration are associated with more severe prognosis. Evidence from controlled studies is limited to mucocutaneous manifestations and eye involvement only. Colchicine is reserved for mild mucocutaneous and/or joint symptoms. Azathioprine and cyclosporine-A have proven efficacy for eye involvement. Cyclophosphamide is mainly utilized for vascular involvement. Anticoagulants may be part of medical treatment of refractory venous thrombosis. Apremilast is an emerging drug that has proven effect against oral and genital ulcerations. Biologic agents have added significantly to our therapeutic repertoire for Behçet syndrome (BS). Especially for severe ocular disease, the introduction of IFN-alpha and TNF-alpha inhibitors led to improvement of visual prognosis. Life-threatening manifestations of BS, such as CNS involvement or pulmonary vasculitis, are managed with TNF-alpha inhibitors when refractory to conventional immunosuppressives. Some preliminary data suggest that biologics other than IFN-alpha and TNF-alpha inhibitors might be useful in the treatment of BS. Both endovascular and open surgical procedures are performed more liberally for the treatment of peripheral and pulmonary aneurysms. Peripheral aneurysms including infrarenal aorta are treated surgically. When the aneurysm is in the suprarenal aorta, hybrid procedures comprising of visceral debranching and endovascular graft insertion lead to low morbidity and mortality. For large pulmonary false aneurysms with abundant hemoptysis, endovascular and surgical interventions can be lifesaving.

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Authors and Affiliations

  1. Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey

    Vedat Hamuryudan

  2. Division of Rheumatology and Systemic Inflammatory Diseases, Department Internal Medicine III, University Hospital Eppendorf (UKE), Hamburg – Clinic for Rheumatology and Immunology, Bad Bramstedt, Germany

    Ina Kötter

  3. Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Cardiovascular Surgery, Istanbul, Turkey

    Hasan Tüzün

Authors
  1. Vedat Hamuryudan
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  2. Ina Kötter
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  3. Hasan Tüzün
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Editor information

Editors and Affiliations

  1. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

  2. Department of Internal Medicine, Istanbul University, Istanbul, Turkey

    Gulen Hatemi

  3. Department of Rheumatology, Istanbul University, Istanbul, Turkey

    Emire Seyahi

  4. Department of Medicine, Academic Hospital, Istanbul, Turkey

    Hasan Yazici

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Hamuryudan, V., Kötter, I., Tüzün, H. (2020). Disease Management. In: Yazici, Y., Hatemi, G., Seyahi, E., Yazici, H. (eds) Behçet Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-24131-5_20

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