Marmara University, School of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey.
Sorbonne Universités, Pitié-Salpêtrière University Hospital, Paris; Department of Internal Medicine and Clinical Immunology, AP-HP, Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose inflammatoire, Paris; Institut National de la Santé et de la Recherche Médicale, INSERM, UMR_S 959, Paris; CNRS, FRE3632, RHU IMAP, Paris, France.
Clin Exp Rheumatol. 2022 Sep;40(8):1491-1496. doi: 10.55563/clinexprheumatol/iovig5. Epub 2022 Feb 2.
Vascular Behçet's disease (VBD) is a systemic vasculitis involving both arterial and venous vessels of all sizes and occurring in up to 40% of patients with BD. VBD is the main cause of mortality in BD. Although commonly seen around the Mediterranean region, comparative studies in VBD are lacking. We aimed to compare the course and therapeutic approaches of VBD in two large cohorts from Turkey and France.
We included 291 VBD patients (female/male:63/228, mean age: 41.2±11.3 years) who were followed up in the Department of Internal Medicine and Clinical Immunology at Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France (n=131) and Rheumatology Division of Marmara University School of Medicine, Istanbul, Turkey (n=160). All clinical and demographical data were acquired from patient charts retrospectively.
Smoking, family history for BD, HLA-B*51 presence and pathergy positivity were significantly higher in Turkish patients (TR), while neurologic involvement was more prominent in the French (FR) group. After a median follow-up of 77 months, 562 vascular events occurred including 440 venous events, 115 arterial events and 7 cardiac thrombi. In 79 (29%) patients, first vascular event developed before BD diagnosis and for 77 (28%) of them, vascular involvement was the presenting sign of the disease. First relapse developed in 130 (44.7%) patients after median 24.5 (1-276) months of follow-up (TR: 46.3% (n=74), FR: 42.7% (n=56), p=0.56). Survival graph revealed that FR cohort has 1.64 times increased recurrent event risk compared to TR cohort (HR=1.64 (1.1-2.44), p=.014) and although did not reach to statistical significance, IS treatment after the first vascular event decreased further vascular events (HR= 0.66 (0.43-1.01, p=.057).
Almost half of patients relapsed of VBD within 2 years after the first vascular event. Immunosuppressants decrease VBD relapses.
血管贝赫切特病（VBD）是一种累及所有大小动脉和静脉的系统性血管炎，发生在多达 40%的贝赫切特病患者中。VBD 是 BD 患者死亡的主要原因。尽管在地中海地区很常见，但 VBD 的比较研究仍很缺乏。我们旨在比较来自土耳其和法国的两个大型队列中 VBD 的病程和治疗方法。
我们纳入了 291 例 VBD 患者（女性/男性：63/228，平均年龄：41.2±11.3 岁），他们在法国巴黎索邦大学皮提-萨尔佩特里埃医院内科和临床免疫学系（n=131）和土耳其伊斯坦布尔马尔马拉大学医学院风湿病科（n=160）接受随访。所有临床和人口统计学数据均从患者病历中回顾性获得。
在土耳其患者（TR）中，吸烟、BD 家族史、HLA-B*51 存在和针刺阳性率显著更高，而法国患者（FR）中神经系统受累更为突出。中位随访 77 个月后，发生了 562 次血管事件，包括 440 次静脉事件、115 次动脉事件和 7 次心脏血栓形成。在 79 名（29%）患者中，首次血管事件发生在 BD 诊断之前，对于其中 77 名（28%）患者，血管受累是疾病的首发表现。在中位随访 24.5（1-276）个月后，130 名（44.7%）患者首次复发（TR：46.3%（n=74），FR：42.7%（n=56），p=0.56）。生存图显示，与 TR 队列相比，FR 队列的复发性事件风险增加了 1.64 倍（HR=1.64（1.1-2.44），p=0.014），尽管没有达到统计学意义，但首次血管事件后使用免疫抑制剂治疗可进一步减少血管事件（HR=0.66（0.43-1.01，p=0.057）。
近一半的 VBD 患者在首次血管事件后 2 年内复发。免疫抑制剂可减少 VBD 复发。
