Disease Management

  • Vedat Hamuryudan
  • Ina Kötter
  • Hasan Tüzün


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.


Vasculitis Azathioprine Colchicine Immunosuppressives Biologic agents TNF-alpha inhibitors Infliximab Adalimumab Interferon alpha EULAR recommendations Vascular surgery False aneurysm Anastomotic aneurysm Ligature of arteries Bypass grafting Endovascular stent Lobectomy 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Vedat Hamuryudan
    • 1
  • Ina Kötter
    • 2
  • Hasan Tüzün
    • 3
  1. 1.Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of RheumatologyIstanbulTurkey
  2. 2.Division of Rheumatology and Systemic Inflammatory Diseases, Department Internal Medicine IIIUniversity Hospital Eppendorf (UKE), Hamburg – Clinic for Rheumatology and ImmunologyBad BramstedtGermany
  3. 3.Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Cardiovascular SurgeryIstanbulTurkey

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