Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample

Silverberg, Jonathan I.; Kwa, Lauren; Kwa, Michael; Laumann, Anne E.; Ardalan, Kaveh

doi:10.1093/rheumatology/kex465

Cited by 38 publications

(34 citation statements)

References 37 publications

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“…Cardiovascular manifestations of juvenile dermatomyositis (JDM), the most common idiopathic inflammatory myopathy of childhood, include acquired structural abnormalities, ventricular dysfunction, arrhythmias, myocarditis, and hypertension [11][12][13][14][15][16][17], with myocardial infarction acting as a rare cause of death [11,13,18,19]. JDM shares the same atherogenic risk factors and vasculopathic features as those of other rheumatologic illnesses, with the added component of lipodystrophy and its related metabolic derangements [15,20,21].…”

Section: Introductionmentioning

confidence: 99%

Assessment of traditional and non-traditional risk factors for premature atherosclerosis in children with juvenile dermatomyositis and pediatric controls

et al. 2020

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Background: Children with juvenile dermatomyositis (JDM), the most common inflammatory myopathy of childhood, may be at increased risk of premature atherosclerosis given a host of traditional and non-traditional risk factors. The primary aim of this study was to determine the underlying frequency of premature atherosclerosis in children with JDM compared to pediatric controls using flow-mediated dilation as a measure of endothelial function. Methods: Children and adolescents with and without JDM were evaluated for traditional atherosclerotic risk factors and assessment of endothelial function, using Endothelial Pulse Amplitude Testing (Endo-PAT). Results: In this study, 75% of pediatric controls were of Black or Hispanic descent (compared to 55% in the JDM group) and 70% were found to live in a household with a medium income less than $50,000/year (compared to 45% in the JDM group). Among traditional atherogenic risk factors, lipoprotein A appeared to be different between controls and JDM patients (66 nmol/L and 16.5 nmol/L, respectively). Using a reactive hyperemia index (RHI) < 1.67 as evidence of endothelial dysfunction, 75% of controls were defined as having endothelial dysfunction compared to 50% in JDM group. When controlled for lipoprotein A as an atherogenic confounder, JDM patients were found to have a 41% increase in RHI, thus indicating less endothelial dysfunction compared to controls. Conclusions: In this study, we have shown that atherogenic risk factors are present in the pediatric population and may be associated with endothelial dysfunction, even at very young ages. Despite increasing concerns that children with rheumatologic disorders may be at increased risk of developing premature atherosclerosis, traditional and sociodemographic features may play a greater role in the ultimate development of cardiovascular disease.

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Section: Introductionmentioning

confidence: 99%

Assessment of traditional and non-traditional risk factors for premature atherosclerosis in children with juvenile dermatomyositis and pediatric controls

et al. 2020

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“…Other manifestations include myocardial infarction, myocarditis, cardiomyopathies, valvular heart disease, pericarditis, pericardial effusion, pulmonary oedema, arrhythmias and conduction block, pulmonary arterial hypertension and venous thromboembolism 23 24. Not only the risk related specifically to the DM (such as myocardial inflammation, endomyocardial fibrosis, small vessel vasculitis, accelerated atherosclerosis),23 but also the traditional risk factors of heart diseases were more prevalent among them 25. As risk of cardiac complications are more common than general population26 27 and the most common cause of death among DM patients,23 they should be evaluated for cardiac comorbidity at the time of diagnosis of myositis 28.…”

Section: Discussionmentioning

confidence: 99%

Dermatomyositis presenting as heart failure revealed a concealed cervical malignancy: an infrequent clinical nexus

et al. 2020

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Dermatomyositis (DM) belongs to the spectrum of inflammatory myopathies which are a heterogeneous group of acquired disorders in which dysregulated immune system is thought to play a major pathologic role. Patients with DM generally present with characteristic skin manifestations with or without muscle weakness and pain. Although subclinical myocardial involvement in DM is well reported in literature, heart failure (HF) as its principal manifestation is extremely rare. Recognition of DM, a known paraneoplastic syndrome, should prompt clinicians to search for underlying malignancy. While gynaecological malignancies are common in association with DM, cervical cancer has rarely been heard of as an aetiology of paraneoplastic DM. We are reporting a case of DM in a postmenopausal woman presenting with advanced decompensated congestive HF with hidden squamous cell carcinoma of cervix.

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“…Interestingly, a small pilot study compared carotid intima-media thickness (CIMT) and flow-mediated dilatation (FMD) as surrogate markers of atherosclerosis in 8 adults with a history of JDM and revealed increased CIMT in JDM patients compared to 8 healthy controls despite the young age ( 37 ). Moreover, recently a large retrospective study demonstrated that JDM was associated with higher odds of cardiovascular and cerebrovascular disease in adolescents, including atherosclerosis, transient ischaemic attacks and cerebral infarction ( 43 ). It is thus possible that the combination of chronic endothelial injury caused by persistent small vessel vasculitis, chronic systemic inflammation, long-term corticosteroid use, sedentary lifestyle, and conventional cardiovascular risk factors predispose patients with JDM to early atherosclerosis.…”

Section: Pathophysiologymentioning

confidence: 99%

The Vasculopathy of Juvenile Dermatomyositis

Papadopoulou

McCann

2018

Front. Pediatr.

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Juvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease. The exact nature of vasculopathy is not yet understood but it is a complex process with both an inflammatory and a non-inflammatory, occlusive component. Impaired function of JDM vasculature includes immune complex deposition, altered expression of cell adhesion molecules predominantly inducing Th17 cell infiltration, and endothelial cell dysfunction. Development of vasculopathy is associated with the severe extra-muscular manifestations of JDM, such as gastrointestinal and cardiac manifestations, interstitial lung disease, ulcerative skin disease or development of calcinosis, and portends a poor prognosis. Correlation of histopathological findings, autoantibodies, and extensive diagnostic workup represent key elements to the early detection of vasculopathic features and early aggressive treatment. Monitoring of vasculopathy remains challenging due to the lack of non-invasive biomarkers. Current treatment approaches provide variable benefit, but better understanding of the essential pathogenic mechanisms should help lead to improved outcomes. Whilst acknowledging that evidence is limited, this review aims to describe the vasculopathy of JDM in the context of pathophysiology, clinical features, and treatment of disease.

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Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample

Abstract: There are significantly higher odds of cardiovascular and cerebrovascular comorbidities among inpatients with JDM, with adolescents, girls and racial/ethnic minorities being at highest risk.

Cited by 38 publications

References 37 publications

Assessment of traditional and non-traditional risk factors for premature atherosclerosis in children with juvenile dermatomyositis and pediatric controls

Assessment of traditional and non-traditional risk factors for premature atherosclerosis in children with juvenile dermatomyositis and pediatric controls

Dermatomyositis presenting as heart failure revealed a concealed cervical malignancy: an infrequent clinical nexus

The Vasculopathy of Juvenile Dermatomyositis

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