Jelušić Marija, Čekada Nastasia, Frković Marijan, Potočki Kristina, Skerlev Mihael, Murat-Sušić Slobodna, Husar Karmela, Đapić Tomislav, Šmigovec Igor, Bajramović Dubravko
Assoc. Prof. Marija Jelušić, MD, PhD Department of Paediatrics Division of Rheumatology and Immunology Referral Centre for Paediatric and Adolescent Rheumatology Republic of Croatia, University Hospital Centre Zagreb University of Zagreb, School of Medicine Kišpatićeva 12, 10000 Zagreb, Croatia;
Acta Dermatovenerol Croat. 2018 Oct;26(3):212-219.
The two most common entities among generally rare but under-diagnosed autoinflammatory bone disorders are chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Due to their similarities, many authors consider CRMO to be a subtype of SAPHO syndrome. The aim of this study was to compare clinical, laboratory, and imaging features and outcomes of patients with CRMO and SAPHO. The analysis of the data from 6 children with CRMO (four girls and two boys, age 3.5-14 years) and of 6 children (6 boys, age 13.5-17.5 years) with SAPHO syndrome was performed. The initiating symptoms in all patients with CRMO were bone pain with multifocal bone lesions. There were no skin manifestations. Five out of six patients achieved control with nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, while one patient required disease-modifying antirheumatic drugs (DMARDs). The initiating symptom in five patients with SAPHO syndrome were severe acne, while in one patient acne occurred two years after the disease onset. Two patients typically developed inflamed sternoclavicular joints and sternum, while the others showed changes affecting other skeletal regions. Three patients achieved control with NSAIDs and corticosteroids, the others required DMARDs and TNFα inhibitors. In comparison with patients with CRMO, patients with SAPHO suffered more frequent and longer lasting exacerbations. In conclusion, CRMO and SAPHO syndrome have an array of common characteristics, but also a number of differences. Nevertheless, further investigation into the etiopathogenesis is required to establish a definite relationship between CRMO and SAPHO.
在通常罕见但诊断不足的自身炎症性骨病中，最常见的两种病症是慢性复发性多灶性骨髓炎（CRMO）和滑膜炎、痤疮、脓疱病、骨肥厚和骨炎（SAPHO）综合征。由于它们的相似性，许多作者认为CRMO是SAPHO综合征的一种亚型。本研究的目的是比较CRMO和SAPHO患者的临床、实验室和影像学特征及预后。对6例CRMO患儿（4名女孩和2名男孩，年龄3.5 - 14岁）和6例SAPHO综合征患儿（6名男孩，年龄13.5 - 17.5岁）的数据进行了分析。所有CRMO患者的起始症状均为伴有多灶性骨病变的骨痛。无皮肤表现。6例患者中有5例通过非甾体抗炎药（NSAIDs）和皮质类固醇实现了病情控制，而1例患者需要使用改善病情的抗风湿药物（DMARDs）。5例SAPHO综合征患者的起始症状为重度痤疮，而1例患者在疾病发作两年后出现痤疮。2例患者典型地出现胸锁关节和胸骨炎症，而其他患者表现为影响其他骨骼区域的改变。3例患者通过NSAIDs和皮质类固醇实现了病情控制，其他患者需要DMARDs和肿瘤坏死因子α抑制剂。与CRMO患者相比，SAPHO患者的病情加重更频繁且持续时间更长。总之，CRMO和SAPHO综合征有一系列共同特征，但也存在一些差异。然而，需要进一步研究病因发病机制以确定CRMO和SAPHO之间的确切关系。
