Ferraioli Mario, Levani Juela, De Luca Riccardo, Matucci-Cerinic Caterina, Gattorno Marco, Guiducci Serena, Bellando Randone Silvia, Chimenti Maria Sole
Rheumatology, Immunology and Clinical Allergology Unit, Department of Medicina dei Sistemi, University of Rome Tor Vergata, 00133 Rome, Italy.
Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, AOU Careggi, 50121 Florence, Italy.
J Clin Med. 2025 Feb 18;14(4):1366. doi: 10.3390/jcm14041366.
Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disease characterized by a sterile inflammatory osteitis and/or arthritis associated with a wide range of dermatological manifestations, such as acne, palmoplantar pustulosis, and psoriasis. This review, providing up-to-date knowledge on this disease, aims at informing researchers and clinicians to help them program future studies in order to improve patients' care. Due to the vast clinical heterogeneity that characterizes this disease, SAPHO syndrome has received various names; among these, chronic recurrent multifocal osteomyelitis represents the most used one. The various nomenclatures in use also reflect different approaches to its management. Indeed, considering the world-wide distribution and the vast onset age (from children to late adulthood), in addition to the multiform clinical presentation, its diagnosis and treatment are often challenging for clinicians. In this review, we provide valuable insights on SAPHO syndrome, delving into its many aspects: epidemiology, pathogenesis, clinical presentation, diagnosis, and classification. Most importantly, this paper addresses the continuously changing treatment panorama of this disease, from established drugs to newly introduced ones. Furthermore, a peculiar focus regards nonpharmacologic approaches, including traditional Chinese medicine, the apheresis technique, and surgery. Similarly, this review also discusses patients' lifestyle, including quality of life. To improve SAPHO syndrome's management, different knowledge gaps should be filled, such as its current epidemiology and pathogenesis. In turn, perfected knowledge in these fields could also advance research in therapy.
滑膜炎-痤疮-脓疱病-骨肥厚-骨炎(SAPHO)综合征是一种罕见疾病,其特征为无菌性炎症性骨炎和/或关节炎,并伴有多种皮肤表现,如痤疮、掌跖脓疱病和银屑病。本综述提供了关于该疾病的最新知识,旨在告知研究人员和临床医生,以帮助他们规划未来的研究,从而改善患者的治疗。由于该疾病具有广泛的临床异质性,SAPHO综合征有多种名称;其中,慢性复发性多灶性骨髓炎是最常用的名称。使用的各种命名法也反映了其不同的治疗方法。事实上,考虑到其全球分布和广泛的发病年龄(从儿童到成年晚期),除了多样的临床表现外,其诊断和治疗对临床医生来说往往具有挑战性。在本综述中,我们对SAPHO综合征提供了有价值的见解,深入探讨了其多个方面:流行病学、发病机制、临床表现、诊断和分类。最重要的是,本文探讨了该疾病不断变化的治疗前景,从已有的药物到新引入的药物。此外,特别关注非药物治疗方法,包括中医、血液分离技术和手术。同样,本综述还讨论了患者的生活方式,包括生活质量。为了改善SAPHO综合征的治疗,应填补不同的知识空白,如其当前的流行病学和发病机制。反过来,这些领域的完善知识也可以推动治疗研究的进展。