Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Vivantes Klinikum Friedrichshain, Children's Hospital, Berlin, Germany.
Curr Osteoporos Rep. 2017 Dec;15(6):542-554. doi: 10.1007/s11914-017-0405-9.
Chronic non-bacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disorder. We summarize the clinical presentation, diagnostic approaches, most recent advances in understanding the pathophysiology, and available treatment options and outcomes in CNO/CRMO.
Though the exact molecular pathophysiology of CNO/CRMO remains somewhat elusive, it appears likely that variable defects in the TLR4/MAPK/inflammasome signaling cascade result in an imbalance between pro- and anti-inflammatory cytokine expressions in monocytes from CNO/CRMO patients. In this context, we present previously unpublished data on cytokine and chemokine expression in monocytes and tissues. CNO/CRMO is an autoinflammatory bone disorder resulting from imbalanced cytokine expression from innate immune cells. Though the exact molecular pathophysiology remains unclear, variable molecular defects appear to result in inflammasome activation and pro-inflammatory cytokine expression in monocytes from CNO/CRMO patients. Recent advances suggest signaling pathways and single molecules as biomarkers for CNO/CRMO as well as future treatment targets.
慢性非细菌性骨髓炎(CNO)及其最严重形式慢性复发性多灶性骨髓炎(CRMO)是一种自身炎症性骨病。我们总结了 CNO/CRMO 的临床表现、诊断方法、对发病机制的最新理解、可用的治疗选择和结果。
尽管 CNO/CRMO 的确切分子发病机制仍有些难以捉摸,但似乎 TLR4/MAPK/炎性小体信号级联的各种缺陷导致 CNO/CRMO 患者单核细胞中促炎和抗炎细胞因子表达失衡。在这方面,我们提供了单核细胞和组织中细胞因子和趋化因子表达的先前未发表的数据。CNO/CRMO 是一种自身炎症性骨病,源于固有免疫细胞中细胞因子表达失衡。尽管确切的分子发病机制尚不清楚,但各种分子缺陷似乎导致 CNO/CRMO 患者单核细胞中的炎性小体激活和促炎细胞因子表达。最近的进展表明信号通路和单个分子可作为 CNO/CRMO 的生物标志物以及未来的治疗靶点。