Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease: A Controlled Study

Objective To assess cardiac function in patients with juvenile mixed connective tissue disease (JMCTD) compared to matched controls, and to investigate possible associations between cardiac impairment and disease variables and cardiovascular risk factors. Methods Fifty JMCTD patients (86% female) examined median 14.9 (6.6–23.0) years after disease onset were compared with 50 age- and sex-matched controls. Electrocardiogram and echocardiography [including eʹ as a marker for diastolic dysfunction and long-axis strain (LAS) and left ventricular (LV) ejection fraction (EF) as markers of systolic function] were performed. LV dysfunction (LVD) was defined as low EF, low LAS, or low eʹ. Right ventricular function was assessed with tricuspid annular plane systolic excursion (TAPSE). Cardiovascular risk factors and disease variables were assessed. Results LVD was found in 16% of patients and 4% of controls (p = 0.035). EF and LAS were lower in patients compared to controls (6% lower, p Conclusion Patients with JMCTD had impaired left and right ventricular function compared to matched controls after median 15 years disease duration. High disease activity and longer treatment with prednisolone were factors associated with LVD.