法国慢性复发性多灶性骨炎患者的大型全国队列研究。

A large national cohort of French patients with chronic recurrent multifocal osteitis.

机构信息

Hôpital Cochin, AP-HP, and Université Paris Descartes, Sorbonne Paris Cité, Paris, France.

出版信息

Arthritis Rheumatol. 2015 Apr;67(4):1128-37. doi: 10.1002/art.39013.

DOI:10.1002/art.39013

PMID:25545761

Abstract

OBJECTIVE

To document more fully the characteristics of chronic recurrent multifocal osteomyelitis (CRMO) in pediatric patients, to collect data on the outcomes and management of the disease, and to define prognostic factors.

METHODS

One hundred seventy-eight patients were included (123 female patients and 55 male patients), with a mean ± SD age at diagnosis of 10.9 ± 2.9 years. Inclusion criteria were a diagnosis of CRMO, evidence of at least one lesion of osteitis confirmed by imaging, and development of the syndrome before age 18 years.

RESULTS

Longitudinal clinical and imaging studies revealed that only 12 of 178 CRMO patients (7%) had unifocal lesions at the last medical visit. We were able to apply the clinical chronic nonbacterial osteomyelitis score to 110 of 178 patients (62%), which indicated that bone biopsy could have been avoided in 27 cases (25%). At the last medical visit, disease was in remission in only 73 of 171 patients (43%) (41% receiving therapy) after a mean ± SD of 47.9 ± 38.9 months; 44 of 171 patients (26%) experienced sequelae. Using cluster analysis, the CRMO cohort was separated into 3 homogeneous phenotypes (severe, mild, and intermediate). Patients with the severe phenotype had the worst prognosis. This group was entirely composed of male patients, most of whom had the multifocal form of CRMO and inflammatory syndrome. Patients with the mild phenotype had the best prognosis. This group was primarily composed of female patients with a unifocal form of CRMO and infrequent clavicle involvement and inflammatory syndrome. Patients with the intermediate phenotype had a good prognosis but greater reliance on treatment. This group primarily included female patients with multifocal lesions and inflammatory syndrome.

CONCLUSION

This is the largest CRMO cohort described in the literature to date. Clinical evolution and imaging investigations confirmed the multifocal pattern of the disease. Three distinct subgroups of CRMO patients were distinguished, with very different prognoses.

摘要

目的

更全面地记录儿童慢性复发性多灶性骨髓炎（CRMO）的特征，收集疾病结局和管理的数据，并确定预后因素。

方法

共纳入 178 例患者（123 例女性患者和 55 例男性患者），诊断时的平均年龄±标准差为 10.9±2.9 岁。纳入标准为 CRMO 诊断、影像学证实至少有一处骨炎病变，以及 18 岁前综合征的发展。

结果

纵向临床和影像学研究显示，178 例 CRMO 患者中仅有 12 例（7%）在最后一次就诊时有单病灶病变。我们能够对 178 例患者中的 110 例（62%）应用临床慢性非细菌性骨髓炎评分，这表明在 27 例（25%）中可以避免骨活检。在最后一次就诊时，171 例患者中仅有 73 例（43%）（41%接受治疗）缓解，平均（±标准差）为 47.9±38.9 个月；171 例患者中有 44 例（26%）出现后遗症。通过聚类分析，CRMO 队列被分为 3 个同质表型（严重、轻度和中度）。严重表型患者预后最差。该组完全由男性患者组成，大多数患者有多发性 CRMO 和炎症综合征。轻度表型患者预后最好。该组主要由女性患者组成，具有单病灶形式，很少涉及锁骨和炎症综合征。中度表型患者预后良好，但更依赖于治疗。该组主要包括女性患者，有多发性病变和炎症综合征。