Groh M, Rogowska K, Monsarrat O, Denoël A, Blanche P, Guillevin L
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.
Clin Exp Rheumatol. 2015 Nov-Dec;33(6):904-5. Epub 2015 Sep 7.
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.
靶向黑色素瘤分化相关基因5(MDA5)编码的核糖核酸解旋酶的自身抗体与临床无肌病性皮肌炎(CADM)相关。明显的全身炎症、皮肤溃疡和严重的间质性肺病似乎很常见。皮肌炎的治疗包括使用免疫抑制剂和/或静脉注射免疫球蛋白,但缺乏循证医学知识。在这种情况下使用阿那白滞素(一种白细胞介素-1受体拮抗剂(IL-1RA))尚未见报道。在此,我们报告一例严重难治性CADM患者使用阿那白滞素后对全身及肌肉外症状(如钙质沉着、关节炎、皮肤溃疡)产生显著快速疗效的病例。不幸的是,短期随访无法评估其对间质性肺病的疗效。IL-1RA可能是难治性CADM的一种有前景的治疗方法。