University of Udine, Italy.
Paediatric Rheumatology, Nephrology, Dermatology Unit, National Reference Centre for Rheumatism and Systemic Autoimmune Diseases in Children RAISE, Hospices Civils de Lyon, France.
Clin Exp Rheumatol. 2020 Nov-Dec;38(6):1255-1262. Epub 2020 Aug 5.
Chronic non-bacterial osteomyelitis (CNO) is a rare non-infectious bone inflammatory disorder; when multifocal, it is referred to as Chronic Recurrent Multifocal Osteomyelitis (CRMO). This study evaluates the demographic, clinical and radiological characteristics of a multi-centre cohort of patients with CNO/CRMO.
Demographic and clinical data of patients with an established diagnosis of CNO/CRMO followed at paediatric rheumatology centres across Europe (Italy, France, Slovenia) and India were retrospectively collected.
There were no demographic differences across countries, but time to diagnosis was significantly longer in India (p=0.041). Pain was almost invariably present at disease onset; functional impairment was more frequent among Italian and Slovenian patients (p=0.001). The number of sites of bone involvement was similar between genders and countries, with long bone metaphises being the most common site. Raised acute phase reactants, detected in >50% of patients, were not associated with clinical manifestations or response to treatment. Comorbidities, evinced in 37% of patients, were equally distributed between genders and nationalities. Imaging approach was similar across countries, without any association between radiological findings and clinical manifestations. NSAIDs were almost invariably used as first-line treatment, but response rate was significantly lower in Italy (p=0.02). Methotrexate was used in 28% of case, with an overall rate of response of 82%. Health conditions and rate of permanent deformities were similar across different countries.
The differences in clinical presentation, radiological features and response to treatment described in this multinational cohort of CNO/CRMO might provide novel insights into this still elusive disease.
慢性非细菌性骨髓炎(CNO)是一种罕见的非传染性骨炎症性疾病;当多灶性时,称为慢性复发性多灶性骨髓炎(CRMO)。本研究评估了多中心队列中 CNO/CRMO 患者的人口统计学、临床和影像学特征。
回顾性收集了在欧洲(意大利、法国、斯洛文尼亚)和印度的儿科风湿病中心就诊的 CNO/CRMO 患者的人口统计学和临床数据。
各国之间没有人口统计学差异,但印度的诊断时间明显更长(p=0.041)。疼痛几乎总是在发病时出现;意大利和斯洛文尼亚患者的功能障碍更为频繁(p=0.001)。骨受累部位的数量在性别和国家之间相似,长骨干骺端是最常见的部位。超过 50%的患者检测到的急性期反应物升高,但与临床表现或治疗反应无关。37%的患者存在合并症,在性别和国籍之间分布均匀。影像学方法在各国之间相似,影像学表现与临床表现之间没有任何关联。非甾体抗炎药几乎总是作为一线治疗,但意大利的反应率明显较低(p=0.02)。28%的病例使用了甲氨蝶呤,总反应率为 82%。不同国家的健康状况和永久性畸形率相似。
本多中心 CNO/CRMO 队列中描述的临床表现、影像学特征和治疗反应的差异可能为这一仍难以捉摸的疾病提供新的见解。