CAJ | 학술논문

目的分析获得性骨肥大( SAPHO)综合征患者的有关临床和影像学表现及其在SAPHO综合征诊断中的作用.方法搜集17例SAPHO综合征患者的临床和影像资料,所有患者均符合下列诊断标准:(1)骨关节病变合并掌跖脓疱或痤疮病变；(2)伴或不伴皮肤病变的弥漫性骨质增生；(3)伴或不伴皮肤病变的慢性复发性多灶性骨髓炎.并均有血清人类白细胞B27型( HLA-B27)抗原检测结果.所有患者均行常规X线摄片检查,13例患者行CT检查,3例患者行MR检查.患者从首诊至最终确诊时间为0.5～13.0年,平均(3.7±1.7)年.结果 17例患者HLA-B27抗原检测结果均为阳性,并均有皮肤病变和骨关节病变.皮肤病变中掌跖脓疱性病变10例,痤疮病变2例,5例确定有皮肤病变,但病史不清.患者骶髂关节均呈不对称受累17例次,胸骨和(或)胸锁关节受累8例次；合并四肢长骨骨髓炎改变5例次.行CT检查的13例,可显示常规X线所不易显示的骨质侵蚀和病变周围的软组织肿胀；行MR检查3例,可显示X线和CT所不能显示的骨髓内水肿样病变.结论虽SAPHO综合征少见,但如患者患有皮肤和骨关节病变,特别是有掌跖脓疱或痤疮的皮肤病变,并有骶髂关节和前胸壁病变的影像特点,诊断上应考虑SAPHO综合征.
목적 분석획득성골비대( SAPHO)종합정환자적유관림상화영상학표현급기재SAPHO종합정진단중적작용.방법 수집17례SAPHO종합정환자적림상화영상자료,소유환자균부합하렬진단표준:(1)골관절병변합병장척농포혹좌창병변；(2)반혹불반피부병변적미만성골질증생；(3)반혹불반피부병변적만성복발성다조성골수염.병균유혈청인류백세포B27형( HLA-B27)항원검측결과.소유환자균행상규X선섭편검사,13례환자행CT검사,3례환자행MR검사.환자종수진지최종학진시간위0.5～13.0년,평균(3.7±1.7)년.결과 17례환자HLA-B27항원검측결과균위양성,병균유피부병변화골관절병변.피부병변중장척농포성병변10례,좌창병변2례,5례학정유피부병변,단병사불청.환자저가관절균정불대칭수루17례차,흉골화(혹)흉쇄관절수루8례차；합병사지장골골수염개변5례차.행CT검사적13례,가현시상규X선소불역현시적골질침식화병변주위적연조직종창；행MR검사3례,가현시X선화CT소불능현시적골수내수종양병변.결론 수SAPHO종합정소견,단여환자환유피부화골관절병변,특별시유장척농포혹좌창 적피부병변,병유저가관절화전흉벽병변적영상특점,진단상응고필SAPHO종합정.
Objective To describe the clinical and imaging manifestations of patients with synovitis acne pustulosis hyperostosis osteomyelitis (SAPHO) syndrome,and to analyze the diagnostic importance of different clinical and imaging manifestations for SAPHO syndrome. Methods Seventeen patients (7 males and 10 females) with SAPHO syndrome were recruited in this study.Age ranged from 36 to 67 years with a mean age of (48 ± 8) years. All patients fulfilled the diagnostic criteria of Benhamou. Serum HLA B27 antigen records were reviewed for all patients. Imaging data of the abnormal bone sites were collected by conventional radiograph in all patients,CT in 13 patients as well as MR in 3 patients.Average time to take for a definite diagnosis of the syndrome was 3.7 years (ranged from O.5 to 13 years).Results Serum HLA B27 antigen was positive in all patients. Both skin and bone abnornalities were found in all patients.Ten patients had skin palmoplantar pustulosis and two patients had acne. Involving sites of bone and joints include sacroiliac joints,anterior chest and limbs.Sacroiliac joints were asymmetrically involved with imaging features in all patients.Eight patients exhibited anterior chest wall involvement. Five patients had osteomyelitis at limbs. For all images of 17 patients,CT was superior to conventional radiography in detecting abnormal changes of bone erosion and soft tissue swelling.MR imaging was able to depict edema changes that was not detectable by CT and radiography.Conclusion SAPHO syndrome is a rare disease,but for patients with skin and bone-joint abnormalities,especially with skin palmoplantar pustulosis,acne as well as with imaging features at the sacroiliac joint and anterior chest wall,SAPHO syndrome should be taken into a diagnostic consideration.