Division of General Pediatrics, Department of Pediatrics and Adolescent Medicine, Medical University Graz, Graz, Austria.
Diagnostic & Research Institute of Pathology, Medical University Graz, Graz, Austria.
Pediatr Rheumatol Online J. 2019 Jul 23;17(1):49. doi: 10.1186/s12969-019-0353-2.
To compare clinical presentation, diagnostic and treatment strategies, and outcome between pediatric and adult patients with chronic non-bacterial osteomyelitis (CNO).
Retrospective single-centre comparative study of pediatric and adult patients diagnosed with chronic recurrent multifocal osteomyelitis (CRMO)/CNO or synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome treated at the Medical University of Graz.
24 pediatric patients diagnosed with CRMO/CNO and 10 adult patients diagnosed with SAPHO syndrome were compared. Median age at diagnosis was 12.3 years (range 7.9-18.9) in the pediatric group and 32.5 years (range 22-56) in the adult group. Median time to diagnosis was shorter in children than in adults (0.3 vs. 1.0 years). Initial clinical presentation, laboratory and histopathological findings were similar in children and adults. Mean numbers of bone lesions were comparable between pediatric and adult patients (3.1 vs. 3.0), as were rates of skin involvement (33% vs. 30%). Sternal involvement was more frequent in adults whereas involvement of clavicle and long bones was more frequent in children (41.7% vs.10, 33% vs. 10%). Computerized tomography (CT) was used more often in adults, whereas whole-body magnetic resonance imaging (MRI) was used only in children. Bisphosphonates were applied more often in children and outcome was better in children than in adults (62.5% vs.30%).
Results of our study suggest that CNO/CRMO and SAPHO syndrome in children and adults might represent a single clinical syndrome that needs a similar diagnostic and therapeutic approach.
比较儿童和成人慢性非细菌性骨髓炎(CNO)患者的临床表现、诊断和治疗策略及结局。
回顾性比较格拉茨医科大学收治的诊断为慢性复发性多灶性骨髓炎(CRMO)/CNO 或滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征的儿童和成人患者。
比较了 24 例诊断为 CRMO/CNO 的儿科患者和 10 例诊断为 SAPHO 综合征的成人患者。儿科组的中位诊断年龄为 12.3 岁(范围 7.9-18.9),成人组为 32.5 岁(范围 22-56)。儿童的确诊时间中位数比成人短(0.3 年比 1.0 年)。儿童和成人的初始临床表现、实验室和组织病理学发现相似。儿科和成人患者的平均骨病变数量相当(3.1 对 3.0),皮肤受累率也相当(33%对 30%)。胸骨受累在成人中更常见,而锁骨和长骨受累在儿童中更常见(41.7%对 10,33%对 10)。成人更常使用计算机断层扫描(CT),而全身磁共振成像(MRI)仅用于儿童。儿童更常使用双膦酸盐,且儿童的结局优于成人(62.5%对 30%)。
我们的研究结果表明,儿童和成人的 CNO/CRMO 和 SAPHO 综合征可能代表一种单一的临床综合征,需要类似的诊断和治疗方法。