Service de Rhumatologie Pédiatrique, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses Inflammatoires (CEREMAIA), Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris (AP-HP), Le Kremlin-Bicêtre, France.
Université Paris-Cité, Paris, France.
Rheumatology (Oxford). 2024 Sep 1;63(SI2):SI233-SI239. doi: 10.1093/rheumatology/kead473.
Extra-osseous (EO) manifestations are poorly characterized in chronic recurrent multifocal osteomyelitis (CRMO). This study aimed to further define the frequency, characteristics and treatment of EO events in CRMO and whether different phenotypes can be distinguished and benefit from special management.
This multicentre retrospective study included CRMO patients followed in several paediatric rheumatology departments in France between 2015 and 2022. EO manifestations were defined as skin lesions, gastrointestinal manifestations, arthritis, enthesitis, sacroiliitis, uveitis, vasculitis and fever. At the last visit, the physician defined CRMO as active in the presence of clinical manifestations including both osseous and EO symptoms.
We included 133 patients; 87 (65.4%) were girls and the median age at first symptoms was 9.0 years (interquartile range 7.0-10.0). EO manifestations were described in 90 (67.7%) patients, with a predominance of skin lesions [n = 51/90 (56.7%)], followed by sacroiliitis [n = 38/90 (42.2%)], enthesitis [n = 21/90 (23.3%)], arthritis [n = 14/90 (15.6%)] and gastrointestinal manifestations [n = 6/90 (6.7%)]. The use of non-steroidal anti-inflammatory drugs and bisphosphonates did not differ by the presence or not of EO manifestations. Biologics were taken more frequently by patients with than without EO manifestations (P < 0.001); TNF inhibitors were used in 33 (36.7%) EO-positive patients. Under this treatment, 18 (54.5%) patients achieved complete remission of osseous and EO manifestations. At the last visit, more EO+ than EO- patients were on treatment (P = 0.009), with active disease in 58 (64.4%) patients.
The analysis of EO manifestations in CRMO delineates two groups of patients in terms of severity and treatments used. Our study opens up new pathophysiological leads that may underlie the wide range of CRMO phenotypes.
在慢性复发性多灶性骨炎（CRMO）中，骨外（EO）表现描述不足。本研究旨在进一步明确 CRMO 中 EO 事件的频率、特征和治疗方法，以及是否可以区分不同的表型并受益于特殊管理。
本多中心回顾性研究纳入了 2015 年至 2022 年期间在法国多个儿科风湿病科就诊的 CRMO 患者。EO 表现定义为皮肤病变、胃肠道表现、关节炎、肌腱炎、骶髂关节炎、葡萄膜炎、血管炎和发热。在最后一次就诊时，医生将有临床症状（包括骨和 EO 症状）的患者定义为活动性 CRMO。
我们共纳入 133 例患者；87 例（65.4%）为女性，首次症状的中位年龄为 9.0 岁（四分位距 7.0-10.0）。90 例（67.7%）患者存在 EO 表现，以皮肤病变为主[51/90（56.7%）]，其次为骶髂关节炎[38/90（42.2%）]、肌腱炎[21/90（23.3%）]、关节炎[14/90（15.6%）]和胃肠道表现[6/90（6.7%）]。非甾体抗炎药和双膦酸盐的使用与是否存在 EO 表现无关。有 EO 表现的患者更常使用生物制剂（P<0.001）；33 例（36.7%）EO 阳性患者使用 TNF 抑制剂。在这种治疗下，18 例（54.5%）患者实现了骨骼和 EO 表现的完全缓解。在最后一次就诊时，与 EO-患者相比，更多的 EO+患者正在接受治疗（P=0.009），58 例（64.4%）患者存在活动性疾病。
CRMO 中 EO 表现的分析表明，在严重程度和治疗方法方面，患者可分为两组。本研究提出了新的病理生理学线索，可能为 CRMO 表型的广泛范围提供依据。
