PDF(Pubmed)
Abstract：
OBJECTIVE: Synovitis acne pustulosis hyperostosis osteitis (SAPHO) is a rare heterogeneous disease of unknown aetiopathology. Externally validated and internationally agreed diagnostic criteria or outcomes and, as a result, prospective randomised controlled trials in SAPHO are absent. Consequently, there is no agreed treatment standard. This study aimed to systematically collate and discuss treatment options in SAPHO.
METHODS: Following \'Preferred Reporting Items for Systematic Reviews and Meta-Analyses\' guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded.
RESULTS: A total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement.
CONCLUSIONS: No single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.
METHODS: Following \'Preferred Reporting Items for Systematic Reviews and Meta-Analyses\' guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded.
RESULTS: A total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement.
CONCLUSIONS: No single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.
摘要：
目的：滑膜炎、痤疮、脓疱性骨炎（SAPHO）是一种少见的异质性疾病，病因不明。经过外部验证和国际商定的诊断标准或结果，因此,SAPHO没有前瞻性随机对照试验。因此,没有商定的治疗标准。本研究旨在系统地整理和讨论SAPHO的治疗方案。
方法：遵循“系统评价和荟萃分析的首选报告项目”指南，使用PubMed进行了系统的文献检索，Scopus和WebofScience数据库。包括前瞻性临床研究和回顾性病例收集，讨论涉及5名或更多参与者的SAPHO的管理和结果。文章不是以英文发表的，未报告明确结果的研究，仅依赖于患者报告结局的研究被排除.
结果：共28项研究（20项观察性，8项开放标签临床研究）报告796名主要为欧洲种族的患者被纳入。报道的疗法差异很大，许多中心并行使用多种治疗。大多数患者（37.1％）单独或联合使用非甾体抗炎药。双膦酸盐（22.1%），常规(21.7%)和生物(11.3%)改善疾病的抗风湿性药物是下一个最常报道的治疗方法.报告的结果各不相同，结果好坏参半，这使得比较复杂化。双膦酸盐表现出最一致的骨关节症状改善，并与短暂的流感样症状有关。在使用TNF抑制剂治疗的患者中报告了矛盾的皮肤反应，但没有严重不良事件记录.大多数治疗对皮肤受累的影响有限或混合。最近一项调查Janus激酶抑制剂tofacitinib的研究在皮肤和指甲受累方面取得了有希望的结果。
结论：目前尚无单一可用的治疗方案足以解决所有SAPHO相关症状。变量,有时描述性结局和治疗组合的使用会使结论和治疗建议复杂化.随机临床试验是必要的，以产生可靠的证据。
方法：遵循“系统评价和荟萃分析的首选报告项目”指南，使用PubMed进行了系统的文献检索，Scopus和WebofScience数据库。包括前瞻性临床研究和回顾性病例收集，讨论涉及5名或更多参与者的SAPHO的管理和结果。文章不是以英文发表的，未报告明确结果的研究，仅依赖于患者报告结局的研究被排除.
结果：共28项研究（20项观察性，8项开放标签临床研究）报告796名主要为欧洲种族的患者被纳入。报道的疗法差异很大，许多中心并行使用多种治疗。大多数患者（37.1％）单独或联合使用非甾体抗炎药。双膦酸盐（22.1%），常规(21.7%)和生物(11.3%)改善疾病的抗风湿性药物是下一个最常报道的治疗方法.报告的结果各不相同，结果好坏参半，这使得比较复杂化。双膦酸盐表现出最一致的骨关节症状改善，并与短暂的流感样症状有关。在使用TNF抑制剂治疗的患者中报告了矛盾的皮肤反应，但没有严重不良事件记录.大多数治疗对皮肤受累的影响有限或混合。最近一项调查Janus激酶抑制剂tofacitinib的研究在皮肤和指甲受累方面取得了有希望的结果。
结论：目前尚无单一可用的治疗方案足以解决所有SAPHO相关症状。变量,有时描述性结局和治疗组合的使用会使结论和治疗建议复杂化.随机临床试验是必要的，以产生可靠的证据。
