Objective
To (i) compare body composition parameters in patients with longstanding juvenile dermatomyositis (JDM) and controls and (ii) explore associations between body composition and disease activity/inflammation, muscle strength, health-related quality of life (HRQL) and cardiometabolic measures.
Methods
In a cross-sectional study, we included 59 patients (median disease duration 16.7 y; median age 21.5 y) and 59 age- and sex-matched controls. Active/inactive disease were defined by the PRINTO criteria. Body composition was assessed by total body dual-energy absorptiometry (DXA), inflammation by hs-CRP and cytokines, muscle strength by manual muscle test (MMT-8), HRQL by 36-item short form survey physical component score (SF-36 PCS) and cardiometabolic function by echocardiography (systolic and diastolic function) and serum-lipids.
Results
DXA analyses revealed lower appendicular lean mass index (ALMI) (reflecting limb skeletal muscle mass), higher body fat percentage (BF%) and higher android: gynoid fat ratio (A: G ratio) (reflecting central fat distribution) in patients than controls, despite similar BMI. Patients with active disease had lower ALMI and higher BF% than those with inactive disease; lower ALMI and higher BF% were associated with inflammation (elevated monocyte attractant protein-1 (MCP-1) and hs-CRP). Lower ALMI was associated with reduced muscle strength; higher BF% was associated with impaired HRQL. Central fat distribution (higher A: G ratio) was associated with impaired cardiac function and unfavorable serum-lipids.
Conclusion
: Despite normal BMI, patients with JDM, especially those with active disease, had unfavorable body composition, which was associated with impaired HRQL/muscle strength and cardiometabolic function. The association between central fat distribution and cardiometabolic alterations is a novel finding in JDM.