gms | German Medical Science

57. Jahrestagung der Deutschen Gesellschaft für Neuropathologie und Neuroanatomie (DGNN)

Deutsche Gesellschaft für Neuropathologie und Neuroanatomie

12. - 15.09.2012, Erlangen

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Hypoxia related molecular mechanisms in adult and juvenile dermatomyositis

Meeting Abstract

  • presenting/speaker Corinna Preuße - Charité - Universitätsmedizin Berlin, Department of Neuropathology, Berlin, Germany
  • Hans-Hilmar Goebel - Charité - Universitätsmedizin Berlin, Department of Neuropathology, Berlin, Germany; Johannes Gutenberg University Mainz, Department of Neuropathology, Mainz, Germany
  • Benedikt Schoser - Klinikum der Universität München, Friedrich-Baur-Institut, Munich, Germany
  • Frank L. Heppner - Charité - Universitätsmedizin Berlin, Department of Neuropathology, Berlin, Germany
  • Ulrike Schara - Universitätsklinikum Essen, 4Department of Neuropediatrics, Essen, Germany
  • Werner Stenzel - Charité - Universitätsmedizin Berlin, Department of Neuropathology, Berlin, Germany

Deutsche Gesellschaft für Neuropathologie und Neuroanatomie. 57th Annual Meeting of the German Society for Neuropathology and Neuroanatomy (DGNN). Erlangen, 12.-15.09.2012. Düsseldorf: German Medical Science GMS Publishing House; 2012. Doc12dgnnPP1.1

DOI: 10.3205/12dgnn019, URN: urn:nbn:de:0183-12dgnn0196

Veröffentlicht: 11. September 2012

© 2012 Preuße et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

The immune-mediated inflammatory myopathies (IIM) comprise the entities dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis (sIBM) and immune-mediated necrotizing myopathy (IMNM)1. The most common form of myopathy in children is juvenile dermatomyositis (jDM). JDM is defined as dermatomyositis with an age of onset below 18 years, and shows an annual incidence of 2–3 per million children.

In addition to the classical symptoms of proximal symmetric muscle weakness further symptoms like arthralgia, fatigue and dysphagia can occur in DM. The specific involvement of the skin is a discriminating factor and can manifest as heliotrope rash of face and décolleté, Gottron's papules and periungual telangiectasias. Elevated CK levels, as well as measurement of auto-immune antibodies can support the diagnosis2.

The aetiology of jDM is unknown and the underlying immune mechanism poorly understood which is why current studies address the question of environmental triggers, genetic predisposition or maternal microchimerism. Furthermore aspects of the immune response, like the role of DCs, are intensively studied2.

On muscle biopsies immune cells can be found in perifascicular infiltrates and apart from the well studied T and B cells one can find a considerable amount of macrophages, which have not been further characterised so far. Additionally molecular signs of hypoxia can be detected focally.

Here we address the question of a possible link between the appearance of inflammatory infiltrates and hypoxia related factors, like HIF-1a, VEGF or STAT3 in jDM and aDM. Therefore we studied muscle biopsies from 10 patients with adult and 12 patients with juvenile DM in comparison to normal controls by diverse histological stainings, immunofluorescence and quantitative real-time PCR. Similarities and differences between juvenile and adult DM are discussed.

Supported by FAZIT Stiftung to CP


References

1.
Hoogendijk JE, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis. Neuromuscul Disord. 2004.
2.
Chiu YE, Co DO. Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use. Pediatr Dermatol. 2011