Treatment Response and Longterm Outcomes in Children with Chronic Nonbacterial Osteomyelitis
Treatment Response and Longterm Outcomes in Children with Chronic Nonbacterial Osteomyelitis
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DOI：
10.3899/jrheum.161255
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发表时间：
2017-07-01
影响因子：
3.9
通讯作者：
Hedrich, Christian M.
中科院分区：
文献类型：
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作者：
Schnabel, Anja;Range, Ursula;Hedrich, Christian M.
目标。慢性非细菌性骨髓炎（CNO）是一种自身炎症性骨疾病，涵盖了广泛的临床范围，从轻度的自限性到慢性活动性或复发性的过程，这些过程被称为慢性复发性多灶性骨髓炎（CRMO）。人们对治疗方案和长期结果知之甚少。我们调查了cno患儿的治疗反应和结局。回顾性图表审查在三级转诊中心进行，涵盖2004-2015年。在治疗开始后的0、3、6、12和24个月以及最后一次有记录的访问时测量疾病活动性。56例CNO患者被确诊；44例为多灶性CNO。50%的患者在中位2.4年后复发，只有40%的患者在5年后没有复发。55例患者使用非甾体类抗炎药作为一线治疗，所有患者3个月后缓解，2年后复发率为50%。进一步治疗包括皮质类固醇（n = 23）、肿瘤坏死因子- α (tnf - α)抑制剂（n = 7）和双膦酸盐（n = 8）。虽然47%的CNO患者在皮质类固醇治疗后1年内复发，但使用tnf - α抑制剂或双膦酸盐（帕米膦酸盐）获得了良好的结果。CNO是一种慢性疾病，第一年预后良好，但长期随访复发率高。特别地，慢性粒细胞白血病患者有长期的、不受控制的炎症，有发展为关节炎的风险。我们的发现强调了及时诊断和开始治疗的重要性。有必要进行前瞻性研究，以建立基于证据的CNO诊断和治疗方法。
Objective. The autoinflammatory bone disorder chronic nonbacterial osteomyelitis (CNO) covers a wide clinical spectrum, ranging from mild self-limited presentations to chronically active or recurrent courses, which are then referred to as chronic recurrent multifocal osteomyelitis (CRMO). Little is known about treatment options and longterm outcomes. We investigated treatment responses and outcomes in children with CNO.Methods. A retrospective chart review was conducted in a tertiary referral center, covering 2004-2015. Disease activity was measured at 0, 3, 6, 12, and 24 months after treatment initiation, and at the last recorded visit.Results. Fifty-six patients with CNO were identified; 44 had multifocal CNO. Fifty percent of patients relapsed after a median of 2.4 years, and as few as 40% remained relapse-free after 5 years. Nonsteroidal antiinflammatory drugs were used as first-line treatment in 55 patients, inducing remission after 3 months in all individuals with relapse rates of 50% after 2 years. Further treatment included corticosteroids (n = 23), tumor necrosis factor-alpha (TNF-alpha) inhibitors (n = 7), and bisphosphonates (n = 8). While 47% of patients with CNO relapsed within 1 year after corticosteroid therapy, favorable outcomes were achieved with TNF-alpha inhibitors or bisphosphonates (pamidronate).Conclusion. CNO is a chronic disease with favorable outcomes within the first year, but high relapse rates in longterm followup. Particularly, patients with CRMO with long-lasting, uncontrolled inflammation were at risk for the development of arthritis. Our findings underscore the importance of a timely diagnosis and treatment initiation. Prospective studies are warranted to establish evidence-based diagnostic and therapeutic approaches to CNO.