Invited Review
Management of vascular Behçet's disease
Fatma Alibaz-Oner,
Haner Direskeneli,
Corresponding Author
Fatma Alibaz-Oner
Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey
Correspondence: Dr Fatma Alibaz-Oner, Division of Rheumatology, Marmara University Hospital, Fevzi Çakmak Mahallesi, Mimar Sinan Cad, No: 41, Ust-Kaynarca, Pendik, Istanbul 34899, Turkey. Email: [email protected]Search for more papers by this authorHaner Direskeneli
Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey
Search for more papers by this authorFatma Alibaz-Oner,
Haner Direskeneli,
Corresponding Author
Fatma Alibaz-Oner
Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey
Correspondence: Dr Fatma Alibaz-Oner, Division of Rheumatology, Marmara University Hospital, Fevzi Çakmak Mahallesi, Mimar Sinan Cad, No: 41, Ust-Kaynarca, Pendik, Istanbul 34899, Turkey. Email: [email protected]Search for more papers by this authorHaner Direskeneli
Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey
Search for more papers by this authorAbstract
Behçet's disease (BD) is a chronic, multisystemic, inflammatory disease characterized by recurrent attacks of mucocutaneous, ocular, musculoskeletal, vascular, central nervous system and gastrointestinal manifestations. Treatment of BD changes according to organ involvement, gender and age of the patient with no golden standard therapeutic regimen. Vascular involvement is observed in up to 40% of the patients with BD, especially in young males and is one of the major causes of mortality and morbidity. Glucocorticoids, azathioprine and cyclophosphamide are still recommended as the first-line treatments in vascular BD. However, increasing data with the tumor necrosis factor inhibitors suggest that these agents may also be acceptable options for the management of refractory vascular BD in daily practice. Anticoagulant usage for vascular BD is also still controversial with limited data coming from retrospective studies. There is a clear need for randomized, controlled studies for the management of VBD.
Conflict of Interest
None declared.
References
- 1Kural-Seyahi E, Fresko I, Seyahi N et al. (2003) The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82, 60–76.
- 2Calamia KT, Schirmer M, Melikoglu M (2011) Major vessel involvement in Behcçet's disease: an update. Curr Opin Rheumatol 23, 24–31.
- 3Yazici H, Yurdakul S, Hamuryudan V (2001) Behçet disease. Curr Opin Rheumatol 13, 18–22.
- 4Sakane T, Takeno M, Suzuki N et al. (1999) Behcet's disease. New Engl J Med 341, 1284–91.
- 5Saadoun D, Wechsler B, Desseaux K et al. (2010) Mortality in Behçet's disease. Arthritis Rheum 62, 2806–12.
- 6Hatemi G, Meara A, Ozguler Y et al. (2017) Developing a core set of outcome measures for Behçet disease: report from OMERACT 2016. J Rheumatol 44, 1750–3.
- 7Galanaud JP, Kahn SR (2013) The postthrombotic syndrome: a 2012 therapeutic update. Curr Treat Options Cardiovasc Med 15, 153–63.
- 8Kahn SR (2009) How I treat postthrombotic syndrome. Blood 114, 4624–31.
- 9Kahn SR, Ducruet T, Lamping DL et al. (2005) Prospective evaluation of health-related quality of life in patients with deep venous thrombosis. Arch Intern Med 165, 1173–8.
- 10Seyahi E, Cakmak OS, Tutar B et al. (2015) Clinical and ultrasonographic evaluation of lower-extremity vein thrombosis in Behcet syndrome: an observational study. Medicine (Baltimore) 94, e189.
- 11Alibaz-Oner F, Aldag B, Aldag M et al. (2016) Post-thrombotic syndrome and venous disease-specific quality of life in patients with vascular Behçet's disease. J Vasc Surg Venous Lymphat Disord 4, 301–6.
- 12Hatemi G, Silman A, Bang DB et al. (2008) EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 67, 1656–62.
- 13Yazici H, Pazarli H, Barnes CG et al. (1990) A controlled trial of azathioprine in Behcet's syndrome. N Engl J Med 322, 281–5.
- 14Hamuryudan V, Ozyazgan Y, Hizli N et al. (1997) Azathioprine in Behcet's syndrome: effects on long-term prognosis. Arthritis Rheum 40, 769–74.
- 15Tascilar K, Melikoglu M, Ugurlu S et al. (2014) Vascular involvement in Behçet's syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford) 53, 2018–22.
- 16Hamuryudan V, Yurdakul S, Moral F et al. (1994) Pulmonary arterial aneurysms in Behcet's syndrome: a report of 24 cases. Br J Rheumatol 33, 48–51.
- 17Hamuryudan V, Er T, Seyahi E et al. (2004) Pulmonary artery aneurysms in Behcet syndrome. Am J Med 117, 867–70.
- 18Seyahi E (2016) Behçet's disease: how to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol 30, 279–95.
- 19Seyahi E, Melikoglu M, Akman C et al. (2012) Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore) 91 (1), 35–48.
- 20Baki K, Villiger PM, Jenni D et al. (2006) Behcet's disease with life-threatening haemoptoe and pulmonary aneurysms: complete remission after infliximab treatment. Ann Rheum Dis 65, 1531–2.
- 21Lee SW, Lee SY, Kim KN et al. (2010) Adalimumab treatment for life threatening pulmonary artery aneurysm in Behc¸et disease: a case report. Clin Rheumatol 29, 91–3.
- 22Chan E, Sangle SR, Coghlan JG et al. (2016) Pulmonary artery aneurysms in Behçet's disease treated with anti-TNFα: a case series and review of the literature. Autoimmun Rev 15, 375–8.
- 23Hamuryudan V, Seyahi E, Ugurlu S et al. (2015) Pulmonary artery involvement in Behcet's syndrome: effects of anti-TNF treatment. Semin Arthritis Rheum 45, 369–73.
- 24Adler S, Baumgartner I, Villiger PM (2012) Behcet's disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res (Hoboken) 64, 607–11.
- 25Vitale A, Emmi G, Lopalco G et al. (2017) Adalimumab effectiveness in Behçet's disease: short and long-term data from a multicenter retrospective observational study. Clin Rheumatol 36, 451–5.
- 26Hibi T, Hirohata S, Kikuchi H et al. (2016) Infliximab therapy for intestinal, neurological, and vascular involvement in Behcet disease: efficacy, safety, and pharmacokinetics in a multicenter, prospective, open-label, single-arm phase 3 study. Medicine (Baltimore) 95, e3863.
- 27Desbois AC, Vallet H, Domont F et al. (2017) Management of severe complications in Behçet's disease with TNF inhibitors. Expert Opin Biol Ther 17(7), 853–9. Epub 2017 May 19.
- 28Cantarini L, Vitale A, Scalini P et al. (2015) Anakinra treatment in drug-resistant Behcet's disease: a case series. Clin Rheumatol 34, 1293–301.
- 29Mohammad AJ, Smith RM, Chow YW et al. (2015) Alemtuzumab as remission induction therapy in Behçet disease: a 20-year experience. J Rheumatol 42, 1906–13.
- 30Desbois AC, Wechsler B, Resche-Rigon M et al. (2012) Immuno-suppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis Rheum 64, 2753–60.
- 31Ozguler Y, Melikoglu M, Cetinkaya F et al. (2012) Behçet sendromunda alt ekstremite akut ven trombozunun prospektif işzlemi. XIII. Turkish National Rheumatolgy Congress. 13–17 October 2012, Antalya, Turkey.
- 32Alibaz-Oner F, Karadeniz A, Yılmaz S et al. (2015) Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Medicine (Baltimore) 94, e494.
- 33Yıldızeli O, Yanartaş M, Taş S et al. (2017) Outcomes of patients with Behçet's syndrome after pulmonary endarterectomy. Thorac Cardiovasc Surg 66, 187–92. [Epub ahead of print].
- 34Tuzun H, Seyahi E, Arslan C et al. (2012) Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease. J Vasc Surg 55, 157e63.
- 35Ozguler Y, Hatemi G (2016) Management of Behçet's syndrome. Curr Opin Rheumatol 28 (1), 45–50.
