Division of Immunology, Department of Internal Medicine.
Division of Pediatric Rheumatology, Allergy and Immunology, Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
Curr Opin Rheumatol. 2020 Sep;32(5):421-426. doi: 10.1097/BOR.0000000000000734.
To describe the main clinical differences of children and adults with chronic nonbacterial osteomyelitis (CNO).
CNO is a severe systemic autoinflammatory syndrome characterized by multiple bone lesions because of inflammatory osteitis. Delay to diagnosis of CNO can lead to functional impairment, fractures, and chronic pain. Key clinical aspects and disease patterns differ in children and adults, including onset and time to diagnosis, symptom localization, associated comorbidities (i.e. skin, joints), bone lesion distribution pattern, and treatment approach. Novel biomarkers, such as urine N-terminal telopeptide in children and serum IgG4 in adults, are being studied for possible future use in improving diagnosis and guiding treatment. Despite recent advances in our understanding of CNO, many children and adults have a high disease burden and poor long-term outcomes. Recent findings suggest that adults with CNO tend to have a more chronic disease course and are less likely to achieve remission in follow-up.
The clinical presentation of CNO differs in children and adults, highlighting the importance of these key features for the accurate diagnosis and early treatment in CNO.
描述儿童和成人慢性非细菌性骨髓炎(CNO)的主要临床差异。
CNO 是一种严重的全身性自身炎症综合征,其特征为炎症性骨炎引起的多处骨骼病变。CNO 的诊断延迟可导致功能障碍、骨折和慢性疼痛。儿童和成人的关键临床方面和疾病模式存在差异,包括发病和诊断时间、症状定位、相关合并症(即皮肤、关节)、骨病变分布模式和治疗方法。正在研究新型生物标志物,如儿童尿 N 端肽和成人血清 IgG4,以提高诊断和指导治疗的可能。尽管我们对 CNO 的认识最近有所提高,但许多儿童和成人仍有较高的疾病负担和较差的长期预后。最近的研究结果表明,CNO 成人患者往往具有更慢性的疾病过程,在随访中不太可能达到缓解。
CNO 的临床表现在儿童和成人中存在差异,突出了这些关键特征对于 CNO 的准确诊断和早期治疗的重要性。