Actuality of juvenile dermatomyositis

Zouagui, Amine; Abourazzak, S.; Idrissi, Mohammed El; Souilmi, Fatima Zohra; Chaouki, S.; Atmani, S.; Bouharrou, A.; Hida, M.

doi:10.1016/j.jbspin.2010.12.007

Cited by 10 publications

(8 citation statements)

References 43 publications

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“…In the juvenile group the female to male ratio was approximately 50:50, while in the aDM group the ratio was 70:30. This result matched data from the literature, indicating an increased proportion of female patients in aDM [ 41 ].…”

Section: Resultssupporting

confidence: 89%

Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis

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Allenbach

Hoffmann

et al. 2016

acta neuropathol commun

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Dermatomyositis (DM) can occur in both adults and juveniles with considerable clinical differences. The links between immune-mediated mechanisms and vasculopathy with respect to development of perifascicular pathology are incompletely understood. We investigated skeletal muscle from newly diagnosed, treatment-naïve juvenile (jDM) and adult dermatomyositis (aDM) patients focusing on hypoxia-related pathomechanisms, vessel pathology, and immune mechanisms especially in the perifascicular region. Therefore, we assessed the skeletal muscle biopsies from 21 aDM, and 15 jDM patients by immunohistochemistry and electron microscopy. Transcriptional analyses of genes involved in hypoxia, as well as in innate and adaptive immunity were performed by quantitative Polymerase chain reaction (qPCR) of whole tissue cross sections including perifascicular muscle fibers.Through these analysis, we found that basic features of DM, like perifascicular atrophy and inflammatory infiltrates, were present at similar levels in jDM and aDM patients. However, jDM was characterized by predominantly hypoxia-driven pathology in perifascicular small fibers and by macrophages expressing markers of hypoxia. A more pronounced regional loss of capillaries, but no relevant activation of type-1 Interferon (IFN)-associated pathways was noted. Conversely, in aDM, IFN-related genes were expressed at significantly elevated levels, and Interferon-stimulated gene (ISG)15 was strongly positive in small perifascicular fibers whereas hypoxia-related mechanisms did not play a significant role.In our study we could provide new molecular data suggesting a conspicuous pathophysiological ‘dichotomy’ between jDM and aDM: In jDM, perifascicular atrophy is tightly linked to hypoxia-related pathology, and poorly to innate immunity. In aDM, perifascicular atrophy is prominently associated with molecules driving innate immunity, while hypoxia-related mechanisms seem to be less relevant.Electronic supplementary materialThe online version of this article (doi:10.1186/s40478-016-0308-5) contains supplementary material, which is available to authorized users.

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Section: Resultssupporting

confidence: 89%

Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis

Preuße

Allenbach

Hoffmann

et al. 2016

acta neuropathol commun

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“…6 Although treatment regimens have substantially improved and mortality rates have significantly lowered, some children still lose their lives to JDM. 7 Diagnosing JDM can be a strenuous process. While 48% of children are ultimately diagnosed with JDM by a pediatric rheumatologist, 8 children often have to consult with multiple physicians before receiving the diagnosis.…”

Section: Introductionmentioning

confidence: 99%

“…As a result, children may experience symptoms of JDM for up to 8.5 months before receiving adequate medical attention. 7 Even if a child seeks medical attention early on, because JDM affects various parts of the body, numerous laboratory and clinical tests are needed to make a formal diagnosis, which can take months. 3…”

Section: Introductionmentioning

confidence: 99%

The family impact of caring for a child with juvenile dermatomyositis

et al. 2017

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Background Juvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin). Despite improvement in treatment regimens and the lowering of mortality rates, some children still lose their lives to JDM. This study assessed the effects of caring for a child diagnosed with JDM on the family system. Methods Participants included 36 mothers and 3 fathers of a child diagnosed with JDM. Parents were administered self-report measures, which assessed the overall family functioning (PedsQL-Family Impact Module), and the parents' mood and level of distress (profile of mood states). Additionally, parents were administered a semi-structured interview that included background information, psychosocial information, and sources of support. Results and conclusion Families of children with JDM reported difficulties in family functioning, communication problems, and an increased number of conflicts. Parents appeared to be experiencing higher than average levels of worry, worse physical functioning, and family relationships when compared to normative populations. Parents would benefit from psychosocial support due to the many challenges associated with caring for a child with JDM.

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“…Juvenile dermatomyositis is the most common of all inflammatory autoimmune myopathies in children, with an estimated incidence of 2-3 cases/million children/year in the United States [4]. The annual incidence rates by race are about 3.4 in Caucasians, 2.7 in Hispanics and 3 in African-American children [4], and it is found twice as commonly in females than in males [5]. In typical cases patients with dermatomyositis seek medical attention when they develop a characteristic rash or muscle weakness.…”

Section: Discussionmentioning

confidence: 99%