Trajectories of cardiorespiratory fitness in patients with juvenile dermatomyositis

Objectives: 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.

Methods: 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.

Results: 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.

Conclusion: 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.