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.
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.
M
M
Groh
Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
K
K
Rogowska
Department of Nuclear Medicine, Hôpital Cochin, Université Paris Descartes, Paris, France.
O
O
Monsarrat
Department of Immunology, Hôpital Bichat, Université Paris Diderot, Paris, France.
A
A
Denoël
Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
P
P
Blanche
Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, (DHU Authors), Inserm U1016, Hôpital Cochin, Université Paris Descartes, Paris, France.
L
L
Guillevin
D018501:Antirheumatic Agents / Q000008:administration & dosage; D015415:Biomarkers / Q000097:blood; D053487:DEAD-box RNA Helicases / Q000276:immunology*; D003882:Dermatomyositis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D016903:Drug Monitoring / Q000379:methods; D005260:Female; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage*; D008875:Middle Aged; D015166:Monitoring, Immunologic; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors*; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D015415:Biomarkers; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1