青年男性以胸痛为首发症状的SAPHO综合征1例并文献复习

A Case of SAPHO Syndrome with Chest Pain as the First Symptom in Young Male and Literature Review

摘要: 目的：SAPHO综合征为临床上缺乏认识、易误诊的自身免疫性非特异性炎症，掌握该病的临床特征，同时结合文献进行讨论，为临床诊治提供参考。方法：分析收治1例青年男性以“胸痛”为首发症状的SAPHO综合征患者，复习相关文献资料，总结临床特征。结果：患者因“右侧胸锁关节疼痛1年余”伴面部、前胸及后背痤疮为主要临床表现收入院，行C反应蛋白(CRP)、红细胞沉降率(ESR)升高，血尿便常规、肝肾功、ENA、抗中性粒细胞胞浆抗体(ANCA)、抗CCP抗体、类风湿因子、免疫球蛋白(Ig)、ANA、HLA-B27未见明显异常；胸部CT平扫：右侧锁骨增粗、硬化，局部见死骨；胸骨柄局部片状骨质硬化；考虑慢性复发性多灶性骨髓炎(自身免疫性疾病，SAPHO综合征)可能性大。骶髂关节CT平扫：双侧骶髂关节间隙可，关节面欠光滑，关节面下可见轻度骨质硬化，强直性脊柱炎不能除外。双侧骶髂关节MR：未见明显异常。全身骨显像：左侧下颌支、双侧锁骨及胸骨柄显像剂浓聚灶，相应CT层面示骨质硬化(右侧锁骨为著)，其中右侧锁骨增粗，考虑SAPHO综合征可能性大。结论：SAPHO综合征是一种以骨、皮肤及关节异常为特征的罕见病，其诊断需根据临床表现、影像学检查，其中全身骨显像对该病的早期诊断具有特异性。早期诊断并及时治疗很重要。

Abstract: Objective: SAPHO syndrome is a kind of autoimmune nonspecific inflammation which is lack of un-derstanding and easy to be misdiagnosed in clinic, so as to grasp the clinical characteristics of the disease and discuss it with reference to the literature, so as to provide reference for clinical diagno-sis and treatment. Methods: A young male patient with SAPHO syndrome with “chest pain” as the first symptom was analyzed, and the related literature was reviewed to summarize the clinical characteristics. Results: The patient was admitted to the hospital because of the main clinical man-ifestations of “pain in the right sternoclavicular joint for more than one year” with acne on the face, front chest and back. The C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were increased. There was no obvious abnormality in blood and urine routine, liver and kidney function, ENA, antineutrophil cytoplasmic antibody (ANCA), anti CCP antibody, rheumatoid factor, immuno-globulin (Ig), ANA, HLA-B27; Chest CT plain scan: the right clavicle is thickened and sclerotic, and dead bone can be seen locally; Partial lamellar osteosclerosis of sternal stalk; It is more likely to consider chronic recurrent multifocal osteomyelitis (autoimmune disease, SAPHO syndrome). Plain CT scan of sacroiliac joint: bilateral sacroiliac joint space is OK, joint surface is not smooth, slight os-teosclerosis can be seen under the joint surface, and ankylosing spondylitis cannot be excluded. MR of bilateral sacroiliac joints: no obvious abnormality is found. Whole body bone imaging: left man-dibular branch, bilateral clavicle and sternal handle imaging agent concentration focus, corre-sponding CT slice shows osteosclerosis (right clavicle is preferred), in which the right clavicle is thickened, so it is considered that SAPHO syndrome is more likely. Conclusion: SAPHO syndrome is a rare disease characterized by abnormal bone, skin and joint. The diagnosis of SAPHO syndrome needs to be based on clinical manifestations and imaging examination, and the whole body bone imaging is specific for the early diagnosis of the disease. Early diagnosis and timely treatment are important.