Blom Kiki J, Takken Tim, Huijgen Barbara C H, Wienke Judith, van Royen-Kerkhof Annet, van Brussel Marco
Child Development and Exercise Centre, Wilhelmina Children's Hospital, University Medical Centre Utrecht.
Centre for Human Movement Sciences, University of Groningen, University Medical Centre Groningen, Groningen.
Rheumatology (Oxford). 2017 Dec 1;56(12):2204-2211. doi: 10.1093/rheumatology/kex366.
Previous research demonstrated decreased cardiorespiratory fitness (CRF) in patients with JDM during active disease and remission. However, longitudinal data regarding trajectories of CRF are currently lacking. The objective of this study was to determine trajectories of CRF in patients with both monocyclic and chronic JDM, and to identify potential predictors of these trajectories.
Thirty-six patients with JDM [median age (interquartile range) at diagnosis: 8.3 (6.3-15.4) years] treated in our paediatric rheumatology outpatient clinic were included. All patients performed multiple cardiopulmonary exercise tests between 2003 and 2016. Relevant CRF parameters were analysed, including peak oxygen uptake, maximal workload, mechanical efficacy and oxygen uptake at ventilatory anaerobic threshold. We analysed trajectories up to 10 years after diagnosis and determined predictors of CRF outcome parameters by multilevel analyses.
Trajectories demonstrated significant declines in CRF during the active phase of the disease with subsequent improvement in CRF during the initial years after diagnosis. However, hereafter no further improvements, and even a decrease, in CRF were observed over time in both monocyclic and chronic subtypes of JDM. We found that a longer disease duration, younger age of onset and higher prednisone dose negatively influence CRF.
Patients with both monocyclic and chronic JDM show decreases in long-term CRF trajectories. Longer disease duration, younger age of onset and higher prednisone dose negatively influence CRF. This study stresses the need for regular evaluation of CRF and implementation of (exercise) interventions to improve CRF in patients with JDM, even in monocyclic patients.
先前的研究表明，幼年皮肌炎（JDM）患者在疾病活动期和缓解期的心肺适能（CRF）均有所下降。然而，目前缺乏关于CRF轨迹的纵向数据。本研究的目的是确定单循环型和慢性JDM患者的CRF轨迹，并识别这些轨迹的潜在预测因素。
纳入在我们儿科风湿病门诊接受治疗的36例JDM患者[诊断时的中位年龄（四分位间距）：8.3（6.3 - 15.4）岁]。所有患者在2003年至2016年间进行了多次心肺运动试验。分析了相关的CRF参数，包括峰值摄氧量、最大工作量、机械效率和通气无氧阈时的摄氧量。我们分析了诊断后长达10年的轨迹，并通过多水平分析确定了CRF结局参数的预测因素。
轨迹显示，疾病活动期CRF显著下降，诊断后的最初几年CRF随后有所改善。然而，此后，在JDM的单循环型和慢性亚型中，随着时间的推移，CRF没有进一步改善，甚至出现下降。我们发现，病程较长、发病年龄较小和泼尼松剂量较高对CRF有负面影响。
单循环型和慢性JDM患者的长期CRF轨迹均下降。病程较长、发病年龄较小和泼尼松剂量较高对CRF有负面影响。本研究强调，即使是单循环型患者，也需要定期评估CRF并实施（运动）干预措施以改善JDM患者的CRF。
