CAJ | 학술논문

目的：分析贝赫切特综合征（Beh?et’s syndrome，BS）合并动脉瘤患者临床特点。方法回顾性分析1997年10月至2014年11月北京协和医院住院的 BS 合并动脉瘤患者临床资料。结果51例患者中共发现94个动脉瘤，其中男性41例，女性10例，从 BS 起病到确诊为动脉瘤中位时间为3．15（0～18．9）年。其中，27例患者（27/51，52．9％）动脉瘤数≥2个。动脉瘤最常发生部位依次为腹主动脉、肺动脉和股动脉，以假性动脉瘤居多。动脉瘤最常见的临床表现是受累动脉相应部位的肿胀和疼痛。合并动脉瘤患者也常合并其他类型血管损害。动脉瘤诊断时，患者红细胞沉降率均值为（35．2±23．4）mm/1 h，C 反应蛋白均值为（40．8±37．9）mg/L，BS 近期活动评分均值为（2．04±0．97）分。51例患者中，38例在诊断动脉瘤后加用大剂量糖皮质激素或激素冲击治疗，43例接受环磷酰胺治疗；20例接受介入治疗（支架植入术、动脉瘤栓塞术），8例行外科手术，1例行支架植入联合外科手术。51例患者住院期间死亡4例，2例死因为大咯血，1例可能为腹主动脉瘤破裂；1例为冠状动脉瘤破裂。结论动脉瘤为 BS 患者的严重并发症，对于 BS 合并动脉瘤患者应警惕多发动脉瘤及合并其他血管损害。治疗上应同时重视原发病病情控制，以及对血管病变的介入或手术处理。
목적：분석패혁절특종합정（Beh?et’s syndrome，BS）합병동맥류환자림상특점。방법회고성분석1997년10월지2014년11월북경협화의원주원적 BS 합병동맥류환자림상자료。결과51례환자중공발현94개동맥류，기중남성41례，녀성10례，종 BS 기병도학진위동맥류중위시간위3．15（0～18．9）년。기중，27례환자（27/51，52．9％）동맥류수≥2개。동맥류최상발생부위의차위복주동맥、폐동맥화고동맥，이가성동맥류거다。동맥류최상견적림상표현시수루동맥상응부위적종창화동통。합병동맥류환자야상합병기타류형혈관손해。동맥류진단시，환자홍세포침강솔균치위（35．2±23．4）mm/1 h，C 반응단백균치위（40．8±37．9）mg/L，BS 근기활동평분균치위（2．04±0．97）분。51례환자중，38례재진단동맥류후가용대제량당피질격소혹격소충격치료，43례접수배린선알치료；20례접수개입치료（지가식입술、동맥류전새술），8례행외과수술，1례행지가식입연합외과수술。51례환자주원기간사망4례，2례사인위대각혈，1례가능위복주동맥류파렬；1례위관상동맥류파렬。결론동맥류위 BS 환자적엄중병발증，대우 BS 합병동맥류환자응경척다발동맥류급합병기타혈관손해。치료상응동시중시원발병병정공제，이급대혈관병변적개입혹수술처리。
Objective To analyze the clinical features of patients with Beh?et’s syndrome(BS)complicated with aneurysmal lesions.Methods We retrospectively reviewed the clinical data of patients with BS complicated with aneurysmal lesions in Peking Union Medical College Hospital from October 1997 to November 2014. Results A total of 94 aneurysms,pseudoaneurysms and dissecting aneurysms were detected in 51 patients,with a male:female ratio of 4.1 ∶1.The median period between BS onset and diagnosis of aneurysmal lesions were 3.15(0-18.9)years.Twenty-seven patients(27/51,52.9%)presented with two or more aneurysmal lesions.The most commonly affected sites were abdominal aorta,pulmonary artery and femoral artery,with a majority of pseudoaneurysms.Swelling and pain in the affected artery area were the major symptoms.Pseudoaneurysms were gengrelly concurrent with other types of vascular involvement.During the onset of aneurysms,erythrocyte sedimentation rate reached (35.2 ±23.4)mm/1 h,C reactive protein levels were (40.8 ±37.9)mg/L,and the BS Current Activity Form 2006 score was (2.04 ±0.97).Large dose of glucocorticoids were administered in 38 patients, and 43 patients were treated with cyclophosphamide.Twenty patients received interventional therapy(graft interposition,graft embolization),8 patients were treated with surgical intervention, and 1 patient had graft interposition with surgical clipping.Four patients died,including 2 patients died with severe hemoptysis,1 patient died with presumed abdominal aneurysm rupture,and the other one for coronary artery rupture.Conclusion Aneurysmal lesions are severe complications in BS patients.BS patients diagnosed with major arterial aneurysms should be further evaluated to detect possible associated vascular involvements or aneurysmal lesions at other sites.We suggest that achieving complete remission of BS and performing surgical or interventional procedures are both important in the treatment of BS patients with aneurysmal lesions.