获得性骨肥大综合征影像表现及其临床诊断价值

Imaging manifestations and its clinical significance in patients with synovitis acne pustulosis hyperostosis osteomyelitis syndrome

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。

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 0. 5 to 13 years）. Results Serum HLA B27 antigen was positive in all patients. Both skin and bone abnormalities were found in all patients. Ten patients had skin palmoplantar pustulosis and two patients had ache. 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, ache as well as with imaging features at the sacroiliac joint and anterior chest wall, SAPHO syndrome should be taken into a diagnostic consideration.