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儿童慢性复发性多灶性骨髓炎:全州三级儿科风湿病转诊中心 9 年经验。

Chronic recurrent multifocal osteomyelitis in children: nine years' experience at a statewide tertiary paediatric rheumatology referral centre.

机构信息

Rheumatology Department, Princess Margaret Hospital for Children, Perth.

Murdoch Childrens Research Institute, Parkville and Department of Paediatrics, University of Melbourne, Victoria, Australia.

出版信息

Rheumatology (Oxford). 2015 Sep;54(9):1688-91. doi: 10.1093/rheumatology/kev013. Epub 2015 May 3.

Abstract

OBJECTIVE

To describe the clinical features, management and outcome of 34 children with chronic recurrent multifocal osteomyelitis (CRMO) diagnosed at a single centre over 9 years.

METHODS

All children identified with CRMO for the period 2005-13 were identified from a prospectively collected database, with additional data from hospital records.

RESULTS

Thirty-four patients, 21 female and 13 male, were identified. The average age at symptom onset was 9.8 years (range 3.8-17.9) and at diagnosis was 10.9 years (range 5.2-18.2), with an average delay in diagnosis of 12 months. Follow-up was 0.3-7.9 years (average 2.1), with 104 individual bony lesions identified, with a median of 3 (range 1-9) per patient. Six patients had unifocal disease. The sites involved included the tibia (n = 19), femur (n = 14), clavicle (n = 12), vertebrae (n = 10) and fibula (n = 8). Approximately half of patients had an inflammatory arthritis at diagnosis, and two-thirds in total eventually developed an arthritis. Pustulosis occurred in eight patients (24%), severe acne in four (12%) and psoriasis in three (9%). NSAIDs were used in 91%, CSs in 82% and MTX in 38%. Two patients were treated with anti-TNF agents. Episodic disease was most common (79%), while 21% had a monophasic pattern. Clinical remission occurred in 94% of children, with prolonged remission in 17%. Seven patients did not require medications for >12 months.

CONCLUSION

CRMO is more common than previously recognized, but diagnosis may be delayed. Episodic multifocal disease was most common, but some had unifocal and/or monophasic disease. Most patients responded to NSAIDs and/or intermittent CSs, but many required DMARDs.

摘要

目的

描述在单一中心经过 9 年的时间诊断出的 34 例慢性复发性多灶性骨髓炎(CRMO)患儿的临床特征、治疗方法和转归。

方法

从一个前瞻性收集的数据库中确定了 2005 年至 2013 年期间患有 CRMO 的所有儿童,同时从医院记录中获取了额外的数据。

结果

共发现 34 名患者,其中 21 名女性,13 名男性。症状发作的平均年龄为 9.8 岁(范围 3.8-17.9),诊断时的平均年龄为 10.9 岁(范围 5.2-18.2),平均诊断延迟 12 个月。随访时间为 0.3-7.9 年(平均 2.1),共发现 104 个单独的骨病变,每个患者的中位数为 3(范围 1-9)。6 例为单灶性疾病。受累部位包括胫骨(n=19)、股骨(n=14)、锁骨(n=12)、椎体(n=10)和腓骨(n=8)。大约一半的患者在诊断时患有炎症性关节炎,而总共三分之二的患者最终发展为关节炎。8 例(24%)患者出现脓疱病,4 例(12%)患者出现严重痤疮,3 例(9%)患者出现银屑病。91%的患者使用了非甾体抗炎药(NSAIDs),82%的患者使用了皮质类固醇(CSs),38%的患者使用了甲氨蝶呤(MTX)。2 例患者使用了抗 TNF 药物。发作性疾病最为常见(79%),而单相型疾病占 21%。94%的患儿临床缓解,17%的患儿缓解持续时间延长。7 例患儿>12 个月无需药物治疗。

结论

CRMO 比以前认为的更为常见,但可能会延迟诊断。发作性多灶性疾病最为常见,但也有一些为单灶性和/或单相性疾病。大多数患者对 NSAIDs 和/或间歇性 CSs 有反应,但许多患者需要 DMARDs。

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