Investigation of possible predictive factors, clinical characteristics, and treatment in vascular Behçet's disease: real-life data from a single center

Yıl 2024, Cilt: 6 Sayı: 3, 281 - 288, 14.10.2024

Abdulvahap Kahveci , Zeycan Kübra Cevval

https://doi.org/10.52827/hititmedj.1442618

Öz

Objective: The aim of this study was to investigate the phenotypes, predictive factors, and treatment approach of Behçet's patients with vascular involvement.
Material and Method: This retrospective study analyzed 123 patients with Behçet's disease, 28 of whom had vascular involvement, and were followed up in our center. The study presented the vascular involvement patterns of the patients along with their clinical characteristics and comorbid conditions. The drugs usage by the patients were analyzed based on the first line and current medications, duration of medical therapy, and drug retention rate.
Results: In Behçet's patients with vascular involvement, the male sex ratio was statistically higher compared to those without vascular involvement (60.7% vs 37.9%; OR=2.82(1.17-6.77); p=0.018). The frequencies of Behçet's clinical manifestations, smoking, and comorbidities were similar in both groups. The most common subtype of vascular Behçet's is deep vein thrombosis (18; 64.2%), followed by superficial thrombophlebitis (5; 17.8%), neurovascular involvement (5; 17.8%), cardio-aortic (2; 7.1%) and pulmonary arterial (2; 7.1%). Azathioprine, glucocorticoid, and cyclophosphamide are the most preferred immunosuppressives in vasculo-Behçet's. Anticoagulant therapy was initiated in 67.8% (19; 28) of the patients at the first vascular event.
Conclusion: The study presented that male gender predicts vascular involvement in Behçet's disease, with deep vein thrombosis being the most common vascular subtype. Although immunosuppressive drugs represent the cornerstone of treatment for vasculo-Behçet's disease, most patients had also received anticoagulant therapy following the initial attack.

Anahtar Kelimeler

anticoagulant, Behçet’s disease, drug retention, immunosuppressive, real-world data, vascular involvement

Vasküler Behçet hastalığında olası prediktif faktörler, klinik karakteristikler ve medikal tedavinin değerlendirilmesi: tek merkezden gerçek yaşam verileri

Öz

Amaç: Bu çalışmanın amacı vasküler tutulumu olan Behçet hastalarının tutulum paternlerini, prediktif faktörleri ve tedavi seçimlerini ortaya koymaktır.
Gereç ve yöntem: Bu çalışmaya merkezimizde takipli 28’i vasküler tutulumlu olan 123 Behçet hastası retrospektif olarak dahil edildi. Hastaların vasküler tutulum paternleri diğer klinik karakteristikleri ve komorbid durumları ile sunuldu. Hastaların kullandıkları ilaçlar ilk başlanılan ajan, mevcut kullanılan ajan, kullanım süresi ve ilaçta kalım değişkenlerine göre analiz edildi.
Bulgular: Vasküler tutulumu olan Behçet hastalarında erkek cinsiyet oranı, vasküler tutulum olmayanlara göre istatistiksel olarak yüksekti (%60,7 vs. %37,9; OR=2,82(1,17-6,77); p=0,018). Her iki grupta Behçet klinik tutulumları, sigara içimi ve komorbidite frekansları benzer bulundu. En sık görülen vasküler Behçet subtipi derin ven trombüsü (18; %64,2) olup onu sırasıyla süperfisyal tromboflebit (5; %17,8), nörovasküler tutulum (5; %17,8), kardiyo-aortik (2; %7,1) ve pulmoner arteriyel tutulum (2; %7,1) izledi. Vasküler Behçet’te en sık tercih edilen immunsupresifler azatioprin, glukokortikoid, siklofosfamid olup antikoagülan tedavi hastaların %67,8 (19; 28)’inde ilk vasküler olayda başlanmıştı.
Sonuç: Bu çalışmada erkek cinsiyetin Behçet hastalığına bağlı vasküler tutulumu predikte ettiği gösterildi. Derin ven trombüsü en sık görülen vasküler Behçet subtipiydi. Vaskülo-Behçet’te immunsupresif ilaçlar ana tedavi olmasına rağmen hastaların büyük bir kısmı ilk atak sonrası antikoagülan tedavi de almıştı.

Anahtar Kelimeler

antikoagülan, Behçet hastalığı, gerçek yaşam verisi, ilaçta kalım, immünosupresif, vasküler etkilenim

Kaynakça

• Hatemi G, Seyahi E, Fresko I, Talarico R, Uçar D, Hamuryudan V. Behçet’s syndrome: one year in review 2022. Clin Exp Rheumatol. 2022;40(8):1461-1471.
• Emmi G, Bettiol A, Silvestri E, et al. Vascular Behçet’s syndrome: an update. Internal and Emergency Medicine. 2019;14:645-652.
• Ishido T, Horita N, Takeuchi M, et al. Clinical manifestations of Behçet’s disease depending on sex and age: results from Japanese nationwide registration. Rheumatology. 2017;56(11):1918-1927.
• Torgutalp M, Sahin Eroglu D, Sezer S, et al. Analysis of vascular involvement in 460 patients with Behçet's syndrome: Clinical characteristics and associated factors. Joint Bone Spine. Mar 2022;89(2):105277.
• Bettiol A, Alibaz-Oner F, Direskeneli H, et al. Vascular Behçet syndrome: from pathogenesis to treatment. Nature Reviews Rheumatology. 2023;19(2):111-126.
• Yazici H, Seyahi E. Behçet syndrome: the vascular cluster. Turkish journal of medical sciences. 2016;46(5):1277-1280.
• Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. Jun 2018;77(6):808-818.
• Alibaz-Oner F, Direskeneli H. Management of vascular Behçet's disease. Int J Rheum Dis. Jan 2019;22 Suppl 1:105-108.
• 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 (Oxford). Nov 2014;53(11):2018-22.
• Fei Y, Li X, Lin S, et al. Major vascular involvement in Behçet's disease: a retrospective study of 796 patients. Clin Rheumatol. Jun 2013;32(6):845-52.
• Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet. May 5 1990;335(8697):1078-80.
• Düzgün N, Ateş A, Aydintuğ O, Demir Ö, Ölmez Ü. Characteristics of vascular involvement in Behçet's disease. Scandinavian journal of rheumatology. 2006;35(1):65-68.
• Ideguchi H, Suda A, Takeno M, Ueda A, Ohno S, Ishigatsubo Y. Characteristics of vascular involvement in Behçet’s disease in Japan: a retrospective cohort study. Clin Exp Rheumatol. 2011;29(4 Suppl 67):S47-53.
• Merashli M, Ster IC, Ames PRJ. Subclinical atherosclerosis in Behcet’s disease: A systematic review and meta-analysis. Elsevier; 2016:502-510.
• Seyahi E, Ugurlu S, Cumali R, et al. Atherosclerosis in Behçet's Syndrome. Seminars in Arthritis and Rheumatism. 2008/08/01/ 2008;38(1):1-12.
• Ugurlu S, Seyahi E, Yazici H. Prevalence of angina, myocardial infarction and intermittent claudication assessed by Rose Questionnaire among patients with Behcet's syndrome. Rheumatology. 2008;47(4):472-475.
• Kaymaz S, Yilmaz H, Furkan U, et al. Ultrasonographic measurement of the vascular wall thickness and intima-media thickness in patients with Behçet’s disease with symptoms or signs of vascular involvement: a cross-sectional study. Archives of Rheumatology. 2021;36(2):258.
• Bettiol A, Hatemi G, Vannozzi L, Barilaro A, Prisco D, Emmi G. Treating the Different Phenotypes of Behçet's Syndrome. Front Immunol. 2019;10:2830.
• Alibaz-Oner F, Karadeniz A, Ylmaz S, et al. Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Medicine. 2015;94(6):e494.
• Desbois A, Wechsler B, Resche‐Rigon M, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis & Rheumatism. 2012;64(8):2753-2760.
• Ozguler Y, Hatemi G, Cetinkaya F, et al. Clinical course of acute deep vein thrombosis of the legs in Behçet’s syndrome. Rheumatology. 2020;59(4):799-806.
• Alibaz-Oner F, Vautier M, Aksoy A, et al. Vascular Behçet’s disease: a comparative study from Turkey and France. Clin Exp Rheumatol. 2022;40(8):1491-1496.