Cardiac dysfunction in juvenile dermatomyositis: a case-control study

T Schwartz,H Sanner,I Sjaastad,T Husebye,B Flato

doi:10.1136/ard.2010.137968

Abstract

ObjectiveTo compare cardiac function in patients with juvenile dermatomyositis (JDM) with matched controls, and examine associations between pathological electrocardiography (ECG), echocardiographic abnormalities and disease variables in patients with JDM.MethodsA total...

Highlights

Juvenile dermatomyositis Juvenile dermatomyositis (JDM) is a rare disease with annual incidence of 1.9 -3.2 /million children reported in population based studies in the UK and US ( 1; 2); still, it is an important systemic connective tissue disease in childhood
We have found systolic and diastolic dysfunction as long term consequences in the disease of JDM; both with associations to long disease duration, high initial disease activity in skin and high cumulative organ damage later in the disease course
High serum levels were associated with long disease duration, high cumulative organ damage and cardiac dysfunction

Summary

Introduction

Juvenile dermatomyositis Juvenile dermatomyositis (JDM) is a rare disease with annual incidence of 1.9 -3.2 /million children reported in population based studies in the UK and US ( 1; 2); still, it is an important systemic connective tissue disease in childhood. Still 30-61% of patients have signs of sustained disease activity and 60-90% develop organ damage 7.2-16.8 after disease onset ( 4; 5; 7). While the mortality rate has decreased ( ,3%) [1], still 30–61% of patients have signs of sustained disease activity and 60–90% develop organ damage 7.2– 16.8 years after disease onset [1,2,3]. Cytokines are small signal molecules, produced by endothelialimmune- and muscle cells They mediate and regulate innate and adaptive immune responses and inflammatory reactions through a number of mechanisms including recruitment and activation of leukocytes [4]. In a controlled study on 37 DM and 19 JDM patients (median disease duration 2 years), several chemokines including monocyte attractant protein-1 (MCP-1) and interferon-inducible protein 10 (IP-10) were increased [9]. Criteria for clinically inactive disease state in JDM were proposed by the Paediatric Rheumatology International Trials Organization (PRINTO) [10]; it is not clear whether disease state is associated with a specific signature of cytokines or inflammatory parameters

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Conclusion