May-Thurner Syndrome as The Presenting Symptoms of Behcet’s Disease: A Rare Case Report
Abstract
Introduction
May-Thurner syndrome as a presenting symptom of Behcet’s disease is rare in the literature. The aim of the current study is to report a rare case of Behcet's disease which has been presented with May-Thurner syndrome.
Case presentation
A 26-year-old woman presented with left leg pain for a one-year duration. On examination, there were dilated superficial veins and edema in the calf. A computed tomography scan of the pelvis showed a markedly narrowed left common iliac vein which was compressed by the right common iliac artery, suggesting May-Thurner syndrome. After a period of not responding to medications, she had been referred to a rheumatologist. The patient was diagnosed with Behcet's disease. She received immunosuppressant agents and glucocorticoids. The patient's symptoms improved after two months of follow-up.
Conclusion
May-Thurner syndrome could be an early manifestation of Behcet's disease, which may further complicate the clinical picture of Behcet's disease.
References
- Tong B, Liu X, Xiao J, Su G. Immunopathogenesis of Behcet's disease. Frontiers in immunology. 2019;10:665.
- doi:10.3389/fimmu.2019.00665
- Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. 2013;65(1):1–11. doi:N/A
- Deuter CM, Kötter I, Wallace GR, Murray PI, Stübiger N, Zierhut M. Behçet's disease: ocular effects and treatment. Progress in retinal and eye research. 2008;27(1):111-36. doi:10.1016/j.preteyeres.2007.09.002
- de Chambrun MP, Wechsler B, Geri G, Cacoub P, Saadoun D. New insights into the pathogenesis of Behcet's disease. Autoimmunity reviews. 2012;11(10):687-98. doi:10.1016/j.autrev.2011.11.026
- Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS. Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clinical rheumatology. 2008;27(2):201-5. doi:10.1007/s10067-007-0685-z
- Alibaz-Oner F, Ergelen R, Mutis A, Erturk Z, Asadov R, Mumcu G, et al. Venous vessel wall thickness in lower extremity is increased in male patients with Behcet’s disease. Clinical rheumatology. 2019;38(5):1447-51. doi:10.1007/s10067-019-04470-z
- Desbois AC, Wechsler B, Resche‐Rigon M, Piette JC, Huong DL, Amoura Z, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis & Rheumatism. 2012;64(8):2753-60. doi:10.1002/art.34450
- Lakha S, Png CY, Chun K, Ting W. Recurrent iliofemoral venous thrombosis in the setting of May-Thurner syndrome as the presenting symptom of Behcet's disease. Annals of vascular surgery. 2018;49:315-e1. doi:10.1016/j.avsg.2017.11.058
- Takeuchi M, Kastner DL, Remmers EF. The immunogenetics of Behçet's disease: A comprehensive review. Journal of autoimmunity. 2015;64:137-48. doi:10.1016/j.jaut.2015.08.013
- Güngen AC, Çoban H, Aydemir Y, Düzenli H. Consider Behcet's disease in young patients with deep vein thrombosis. Respiratory medicine case reports. 2016;18:41-4. doi:10.1016/j.rmcr.2016.04.002
- Saadoun D, Wechsler B, Desseaux K, Huong DL, Amoura Z, Resche‐Rigon M, et al. Mortality in Behçet's disease. Arthritis & Rheumatism. 2010;62(9):2806-12. doi:10.1002/art.27568
- Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and ultrasonographic evaluation of lower-extremity vein thrombosis in Behcet syndrome: an observational study. Medicine. 2015;94(44). doi:10.1097/MD.0000000000001899
- Gurunathan A, Teachey DT, Chikwava KR, Witmer C, Desai AV. Behcet disease initially presenting as deep venous thrombosis: a case report. Journal of Pediatric Hematology/Oncology. 2017;39(5):410-2. doi:10.1097/MPH.0000000000000830
- de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA–B51/B5 and the risk of Behçet's disease: A systematic review and meta‐analysis of case–control genetic association studies. Arthritis Care & Research. 2009;61(10):1287-96.
- doi:10.1002/art.24642
- Blake T, Pickup L, Carruthers D, Damato EM, Denniston A, Hamburger J, et al. Birmingham Behçet’s service: classification of disease and application of the 2014 International Criteria for Behçet’s Disease (ICBD) to a UK cohort. BMC Musculoskeletal Disorders. 2017;18(1):1-8. doi:10.1186/s12891-017-1463-y
- Kirino Y, Ideguchi H, Takeno M, Suda A, Higashitani K, Kunishita Y, et al. Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet’s disease: a retrospective observational study. Arthritis research & therapy. 2016;18(1):1-8. doi:10.1186/s13075-016-1115-x
- International Team for the Revision of the International Criteria for Behçet's Disease (ITR‐ICBD), Davatchi F, Assaad‐Khalil S, Calamia KT, Crook JE, Sadeghi‐Abdollahi B, Schirmer M, et al. The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. Journal of the european Academy of Dermatology and Venereology. 2014;28(3):338-47. doi:10.1111/jdv.12107
- Akiyama M, Kaneko Y, Takeuchi T. Effectiveness of tocilizumab in Behcet's disease: a systematic literature review. InSeminars in Arthritis and Rheumatism 2020;50(4):797-804. doi:10.1016/j.semarthrit.2020.05.017
- Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, et al. Management of Behçet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet disease. Annals of the rheumatic diseases. 2009;68(10):1528-34. doi:10.1136/ard.2008.087957
- Alibaz-Oner F, Direskeneli H. Advances in the Treatment of Behcet’s Disease. Current rheumatology reports. 2021;23(6):1-0. doi:10.1007/s11926-021-01011-z
- Alibaz‐Oner F, Direskeneli H. Management of vascular Behcet's disease. International Journal of Rheumatic Diseases. 2019;22:105-8. doi:10.1111/1756-185X.13298
- Hamuryudan V, Özyazgan Y, Hizli N, Mat C, Yurdakul S, Tüzün Y, et al. Azathioprine in Behçet's syndrome. Effects on long‐term prognosis. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology. 1997;40(4):769-74. doi:10.1002/art.1780400425
- Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course. Rheumatology. 2014;53(11):2018-22. doi:10.1093/rheumatology/keu233
- Alibaz-Oner F, Karadeniz A, Yılmaz S, Balkarlı A, Kimyon G, Yazıcı A, et al. Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Medicine. 2015;94(6). doi:10.1097/MD.0000000000000494
- Alibaz-Oner F, Aldag B, Aldag M, Unal AU, Mutis A, Toptas T, et al. AB0540 Post-Thrombotic Syndrome and Venous Disease Spesific Quality of Life in Patients with Vascular Behçet's Disease. Annals of the Rheumatic Diseases.2016;75(2):1090-1090. doi:10.1136/annrheumdis-2016-eular.1238
- Han SW, Kim GW, Lee J, Kim YJ, Kang YM. Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet’s disease. Rheumatology international. 2005;25(3):234-7. doi:10.1007/s00296-004-0495-4
- Tekbas G, Oguzkurt L, Gur S, Onder H, Andic C. Endovascular treatment of veno-occlusive Behcet’s disease. Cardiovascular and interventional radiology. 2012;35(4):826-31.
- doi:10.1007/s00270-011-0322-8
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.