Interleukin-1 receptor antagonist for refractory anti-MDA5 clinically amyopathic dermatomyopathy.


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.

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@article{Groh2015Interleukin1RA, title={Interleukin-1 receptor antagonist for refractory anti-MDA5 clinically amyopathic dermatomyopathy.}, author={Matthieu Groh and Katarzyna Rogowska and O Monsarrat and Albert Deno{\"{e}l and Philippe Blanche and L Guillevin}, journal={Clinical and experimental rheumatology}, year={2015}, volume={33 6}, pages={904-5} }