Department of Rheumatology and Autoimmunology, Shandong Provincial Qianfoshan Hospital, Cheeloo College of Medicine, 12589Shandong University, Shandong University, Jinan, Shandong, P. R. China.
Cheeloo College of Medicine, 12589Shandong University, Shandong University, Jinan, Shandong, P. R. China.
J Int Med Res. 2021 Dec;49(12):3000605211065314. doi: 10.1177/03000605211065314.
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations, but has rarely been clarified in the clinic. We report a 28-year-old woman who had initial onset of SAPHO syndrome with involvement of the femur, and she experienced a tortuous diagnostic course. We also performed a literature review of SAPHO syndrome cases involving the femur and summarize several empirical conclusions by integrating previous findings with our case. Furthermore, we propose our perspective that ailment of the skin caused by infection of pathogens might be the first hit for triggering or perpetuating the activation of the immune system. As a result, musculoskeletal manifestations are probably the second hit by crosstalk of an autoimmune reaction. The skin manifestations preceding bone lesions can be well explained. Current interventions for SAPHO syndrome remain controversial, but drugs aiming at symptom relief could serve as the first preference for treatment. An accurate diagnosis and appropriate treatment can cure patients in a timely manner. Although the pathogenesis of SAPHO syndrome remains to be determined, physicians and surgeons still need to heighten awareness of this entity to avoid invasive procedures, such as frequent biopsies or nonessential ostectomy.
滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(SAPHO)综合征是一个涵盖一系列骨骼病变和皮肤表现的伞状术语,但在临床上很少得到明确。我们报告了一例 28 岁女性,最初患有 SAPHO 综合征,累及股骨,并经历了曲折的诊断过程。我们还对涉及股骨的 SAPHO 综合征病例进行了文献回顾,并将以前的发现与我们的病例相结合,总结了一些经验性结论。此外,我们提出了这样的观点,即病原体感染引起的皮肤疾病可能是触发或维持免疫系统激活的第一击。因此,骨骼肌肉表现可能是自身免疫反应的第二击。骨骼病变前的皮肤表现可以很好地解释。目前 SAPHO 综合征的干预措施仍存在争议,但针对症状缓解的药物可能是治疗的首选。准确的诊断和适当的治疗可以及时治愈患者。虽然 SAPHO 综合征的发病机制仍有待确定,但医生和外科医生仍需提高对该疾病的认识,以避免频繁的活检或不必要的骨切除术等有创性操作。