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Connective tissue diseases
Clinical case
Dermatomyositis flare on imiquimod therapy highlights a crucial role of aberrant TLR7 signalling
  1. Alain Meyer1,2,3,
  2. Ghada Alsaleh3,4,
  3. Claude Heuschling5,
  4. Jacques-Eric Gottenberg2,3,
  5. Philippe Georgel3,4,
  6. Benard Geny2,3,
  7. Seiamak Bahram3,4 and
  8. Jean Sibilia2,3,4
  1. 1Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
  2. 2Nouvel Hôpital Civil, Service des Explorations Fonctionnelles, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
  3. 3Fédération de Médecine Translationnelle (FMTS), Strasbourg, France
  4. 4Laboratoire d'ImmunoRhumatologie Moléculaire, INSERM UMR_S1109, Centre de Recherche d'Immunologie et d'Hématologie, Faculté de Médecine, Université de Strasbourg, Strasbourg, France
  5. 5Cabinet de Rhumatologie, Esch-sur-Alzette, Luxembourg
  1. Correspondence to Dr Alain Meyer; alain.meyer1{at}chru-strasbourg.fr

https://doi.org/10.1136/rmdopen-2016-000294

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    Key messages

    What is already known about this subject?

    • It has been speculated that elevated interferon (IFN)-β in blood characterising dermatomyositis patients results from the engagement of endosomal toll-like receptor (TLR) signalling due to the increased expressions of TLR7 and TLR9 in peripheral blood leucocytes.

    What does this study add?

    • We provide direct evidence that supports this hypothesis by reporting herein a patient who developed severe exacerbation of anti-NXP2-positive dermatomyositis (DM) on imiquimod therapy, a potent TLR7 agonist and whose peripheral blood mononuclear cells secreted high level of IFN-β upon TLR7 stimulation as compared with healthy donors.

    How might this impact on clinical practice?

    • These data indicate that aberrant TLR7 signalling may represent a therapeutic target in DM.

    Dermatomyositis (DM) is a chronic systemic disease that primarily affects skin and/or muscles and is associated with cancer in about 20% of cases. Although DM is an autoimmune disorder, there is evidence that innate immunity plays a crucial role in the disease. In particular, it has been associated with elevated interferon (IFN)-β in blood1 which is critical in the initiation2 and perpetuation3 of the disease. However, the origin of elevated IFN-β remains elusive. It has been speculated that it may result from the engagement of endosomal toll-like receptor (TLR) signalling due to the increased expressions of TLR7 and TLR9 in peripheral blood leucocytes4 of patients with DM, but direct evidence of endosomal TLR involvement in DM is lacking.

    We report herein …

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