Case Reports
Interleukin-1 receptor antagonist for refractory anti-MDA5 clinically amyopathic dermatomyopathy
M. Groh1, K. Rogowska2, O. Monsarrat3, A. Denoël4, P. Blanche5, L. Guillevin6
- Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France. matthieu.groh@cch.aphp.fr
- Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
- Department of Nuclear Medicine, Hôpital Cochin, Université Paris Descartes, Paris, France.
- Department of Immunology, Hôpital Bichat, Université Paris Diderot, Paris, France.
- Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
- Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
CER8401
2015 Vol.33, N°6
PI 0904, PF 0905
Case Reports
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PMID: 26343354 [PubMed]
Received: 25/02/2015
Accepted : 04/05/2015
In Press: 07/09/2015
Published: 15/12/2015
Abstract
Autoantibodies targeting the melanoma-differentiation-associated gene-5 (MDA5)-encoded ribonucleic acid helicase are associated with clinically amyopathic dermatomyopathy (CADM). Marked systemic inflammation, skin ulcers and severe interstitial lung disease seem frequent. DM treatment consists of immunosuppressants and/or intravenous immunoglobulins, but evidence-based knowledge is lacking. Anakinra (an interleukin-1 receptor antagonist (IL-1RA)) use in this setting has never been reported. Herein, we report on a case of anakinra dramatic and rapid efficacy against general and extramuscular (e.g. calcinosis, arthritis, skin ulcers) in a patient with severe and refractory CADM. Unfortunately, short-term follow-up prevented efficacy evaluation against interstitial lung disease. IL-1RA could be a promising treatment for refractory CADM.
