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Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update.
Therapeutic Advances in Musculoskeletal Disease ( IF 3.4 ) Pub Date : 2020-05-12 , DOI: 10.1177/1759720x20912865
Shuang Liu 1 , Mingwei Tang 1 , Yihan Cao 2 , Chen Li 3
Affiliation  

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a spectrum of heterogeneous diseases characterized by osteoarticular and dermatological manifestations. Osteitis and hyperostosis are core clinical manifestations in SAPHO syndrome, typically affecting multiple areas and possibly progressing to irreversible osteoarticular damage. Most patients with SAPHO have cutaneous involvement, mainly manifested as palmoplantar pustulosis and severe acne. Systemic manifestations are uncommon but occasionally reported. Epidemiological studies suggest the annual prevalence of SAPHO syndrome varies from 0.00144 in 100,000 in Japanese individuals to fewer than 1 in 10,000 in White individuals. The precise etiopathogenesis of SAPHO remains unclear, but it is generally considered an autoinflammatory syndrome that may be related to various etiologies, such as immune dysfunction, infection and genetic predisposition. Owing to the relapsing–remitting disease course, the goal of management is to improve clinical symptoms and prevent disease progression. Various treatments, including nonsteroidal anti-inflammatory drugs, conventional disease-modifying antirheumatic drugs, bisphosphonates, biologics, and antibiotics, are promising options for alleviating the disease.



中文翻译:

滑膜炎,痤疮,脓疱病,骨肥厚和骨炎综合征:复习和更新。

滑膜炎,痤疮,脓疱病,肥大和骨炎(SAPHO)综合征是一系列异质性疾病,其特征是骨关节和皮肤病学表现。骨炎和骨质增生是SAPHO综合征的核心临床表现,通常影响多个区域,并可能发展为不可逆的骨关节损伤。SAPHO的大多数患者都有皮肤受累,主要表现为掌plant脓疱病和严重的痤疮。全身表现不常见,但偶尔有报道。流行病学研究表明,SAPHO综合征的年患病率从日本人的100,000的0.00144变化到白人不到10,000的1。SAPHO的确切病因尚不清楚,但通常被认为是一种自身炎症综合症,可能与各种病因有关,例如免疫功能障碍,感染和遗传易感性。由于病情不断复发,管理的目标是改善临床症状并预防疾病进展。各种治疗方法,包括非甾体类抗炎药,常规改变疾病的抗风湿药,双膦酸盐,生物制剂和抗生素,都是缓解疾病的有前途的选择。

更新日期:2020-05-12
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