32例血管型白塞病的临床特征分析

An Analysis of Clinical Characteristics in 32 Cases of Vascular Behcet’s Disease

目的分析总结血管型白塞病(Behcet’s disease,BD)患者的临床特征、影像学改变、治疗及预后。方法收集整理并分析首都医科大学附属北京世纪坛医院于2007年1月至2021年11月收治的血管型BD患者的一般情况、临床表现、实验室检查、影像学改变、治疗及预后情况。结果共收治血管型BD患者32例,男性25例,女性7例,平均发病年龄36.8岁,平均发病时间约为52.4月。32例患者均有口腔溃疡,21例合并生殖器溃疡;18例患者针刺反应阳性;12例患者累及眼部;4例患者出现胸闷等不同程度的呼吸困难、肿胀及颈胸部静脉扩张;4例患者出现肝脾肿大、腹腔积液。大部分患者血沉、C反应蛋白及血常规白细胞计数升高。32例患者中有21例患者单纯累及静脉,3例患者单纯累及动脉,8例患者动脉及静脉均有受累;29例累及静脉的患者中,24例患者累及下肢深静脉,1例患者合并胸导管梗阻并乳糜胸形成,4例患者累及上腔静脉并出现上腔静脉综合征,4例患者累及肝静脉或肝上段的下腔静脉并出现布加综合征。32例患者均应用糖皮质激素联合免疫抑制剂,其中21例患者应用环磷酰胺,18例应用沙利度胺,11例应用秋水仙碱,5例应用生物制剂英夫里昔单抗;17例患者联合应用抗凝药物治疗;5例患者行手术治疗。经随访,大部分患者病情稳定,8例患者病情明显好转,1例患者死于动脉瘤破裂。结论血管型BD好发青年男性,起病隐匿,动静脉均可受累,以下肢深静脉受累最常见,部分患者症状严重,临床医生应提高警惕,早期联合用药可改善患者预后。

Objective To analyze and summarize the clinical features,treatment and prognosis of vascular Behcet’s disease(BD)patients.Methods The general information,clinical manifestations,laboratory test results,imaging changes,treatment and prognosis of patients with vascular BD admitted to Beijing Shijitan Hospital affiliated to Capital Medical University from January,2007 to November,2021 were collected and analyzed.Results A total of 32 patients with vascular BD were treated,including 25 males and 7 females.The mean age of onset was 36.8 years,and the mean time of onset was about 52.4 months.All 32 patients had a history of oral ulcer,21 patients of whom were with genital ulcer.18 patients had positive reaction to acupuncture.Ocular involvement was observed in 12 patients.Four patients had chest tightness and other varying degrees of dyspnea,swelling and jugular chest vein dilatation.Hepatosplenomegaly and peritoneal effusion were observed in 4 patients.Erythrocyte sedimentation rate,C-reactive protein and white blood cell count increased in most patients.Among 32 patients,21 patients had simple venous involvement,3 patients had simple arterial involvement,and 8 patients had both arterial and venous involvement.Among 29 patients with venous involvement,24 patients also involved with deep lower extremity veins,1 patient complicated with thoracic duct obstruction and chylothorax formation,4 patients involved with superior vena cava and developed superior vena cava syndrome,and 4 patients involved with hepatic vein or superior hepatic inferior vena cava and developed Budd-Chiari syndrome.All 32 patients were treated with glucocorticoids combined with immunosuppressive agents,including cyclophosphamide(n=21),thalidomide(n=18),colchicine(n=11)and infliximab(n=5).17 patients were treated with anticoagulant drugs.Five patients underwent surgical treatment.After follow-up,most of the patients’conditions were stable,while 8 patients’condition improved significantly and 1 patient died of aneurysm rupture.Conclusion Vascular BD tends to occur in young men,with insidiously onset,and can be involved in both arteries and veins,especially in lower extremity deep veins.Some patients have severe symptoms.Clinicians should be vigilant,and early combined medication can improve the prognosis of patients.