How Often are Pediatric Patients with Clinically Amyopathic Dermatomyositis Truly Amyopathic?

Oberle, Edward J; Bayer, Michelle L.; Chiu, Yvonne E.; Co, Dominic O.

doi:10.1111/pde.13013

Cited by 13 publications

(18 citation statements)

References 15 publications

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“…In addition, we found that CAJDM patients have less frequent myalgias, arthralgias and fatigue than JDM patients, and none of our patients developed arthritis, in contrast to other reports [3]. We found a similar distribution in the cutaneous manifestations of CAJDM and JDM patients, as reported by others [1,6]. Importantly, calcinosis, vasculitis and lipodystrophy were absent in CAJDM patients, also as previously reported [1,6], although calcinosis was uncommonly seen in one series of CAJDM patients [3].…”

Section: Discussionsupporting

confidence: 86%

“…In adults, CADM represents 1020% of the DM population [1,5]. In children, CAJDM constituted 22% of patients with JDM in one clinic population [6], although relatively few cases of CAJDM in other reports suggests that the frequency may be lower in children [3,4]. Melanoma differentiation-associated gene 5 (MDA5) autoantibodies are associated with amyopathic DM and rapidly progressing lung disease in Asian juvenile and Caucasian adult DM patients [710].…”

Section: Introductionmentioning

confidence: 99%

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Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

et al. 2018

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Section: Discussionsupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

et al. 2018

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“…True amyopathic JDM however is very rare and mild muscle involvement may be present but missed (13). Amyopathic JDM generally has a relatively mild disease course with fewer systemic manifestations, less required immunosuppressive treatment and a good prognosis (12, 14, 15).…”

Section: Signs Of Systemic Disease Activity In Jdm Based On Affected mentioning

confidence: 99%

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

et al. 2018

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Juvenile Dermatomyositis (JDM) is a systemic immune-mediated disease of childhood, characterized by muscle weakness, and a typical skin rash. Other organ systems and tissues such as the lungs, heart, and intestines can be involved, but may be under-evaluated. The inflammatory process in JDM is characterized by an interferon signature and infiltration of immune cells such as T cells and plasmacytoid dendritic cells into the affected tissues. Vasculopathy due to loss and dysfunction of endothelial cells as a result of the inflammation is thought to underlie the symptoms in most organs and tissues. JDM is a heterogeneous disease, and several disease phenotypes, each with a varying combination of affected tissues and organs, are linked to the presence of myositis autoantibodies. These autoantibodies have therefore been extensively studied as biomarkers for the disease phenotype and its associated prognosis. Next to identifying the JDM phenotype, monitoring of disease activity and disease-inflicted damage not only in muscle and skin, but also in other organs and tissues, is an important part of clinical follow-up, as these are key determinants for the long-term outcomes of patients. Various monitoring tools are currently available, among which clinical assessment, histopathological investigation of muscle and skin biopsies, and laboratory testing of blood for specific biomarkers. These investigations also give novel insights into the underlying immunological processes that drive inflammation in JDM and suggest a strong link between the interferon signature and vasculopathy. New tools are being developed in the quest for minimally invasive, but sensitive and specific diagnostic methods that correlate well with clinical symptoms or reflect local, low-grade inflammation. In this review we will discuss the types of (extra)muscular tissue inflammation in JDM and their relation to vasculopathic changes, critically assess the available diagnostic methods including myositis autoantibodies and newly identified biomarkers, and reflect on the immunopathogenic implications of identified markers.

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“…Some have questioned the concept of CADM with the argument that if an aggressive search for muscle inflammation is carried out [i.e., all five muscle enzymes (aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, creatine kinase and aldolase)], as well as magnetic resonance imaging and muscle biopsy), virtually all juvenile-onset CADM patients will be found to have "subclinical" evidence of myositis (9). (Such patients would meet the criteria for the hypomyopathic subtype of CADM).…”

Section: Clinically-amyopathic Dmmentioning

confidence: 99%

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

Sontheimer¹

2017

Annals of Translational Medicine

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Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i.e., "clinically-amyopathic DM" (CADM)] and were at risk for acute, rapidly-progressive form of interstitial lung disease (ILD). As an academic dermatologist in the United States of America (USA) having a career-long interest in the CADM subset, I would like to share my perspective on the results of the work by Allenbach and colleagues and offer some suggestions for additional study in this area. But to do so most effectively, I first would like to review the clinical concept of CADM and its association with anti-MDA5 antibody production and a potentially-fatal form of (ILD).

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How Often are Pediatric Patients with Clinically Amyopathic Dermatomyositis Truly Amyopathic?

Abstract: These data suggest that truly amyopathic JDM is rare and that a thorough workup that includes all five muscle enzymes and MRI may uncover occult myositis.

Cited by 13 publications

References 15 publications

Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

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