University of Southern California Keck School of Medicine, Los Angeles, California, USA.
Division of Rheumatology, University of Southern California, Los Angeles, California, USA
BMJ Case Rep. 2020 Sep 30;13(9):e236431. doi: 10.1136/bcr-2020-236431.
Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.
抗黑色素瘤分化相关基因 5（MDA-5）皮肌炎是皮肌炎的一种亚型，与快速进展性间质性肺病（RP-ILD）以及多种皮肤表现相关。MDA-5 皮肌炎患者的预后往往较差，这通常归因于并发 RP-ILD 的高发生率。鉴于疾病的严重程度，早期诊断和积极治疗至关重要。我们报告了一例 40 岁女性，诊断为 MDA-5 皮肌炎，表现为乏力、疼痛性皮肤溃疡和间质性肺病。她接受了每月静脉注射免疫球蛋白（IVIg）、基于体重的泼尼松和霉酚酸酯（MMF）治疗。经过大约 2 年的治疗，她的间质性肺病仍然稳定，乏力和皮肤溃疡有了显著改善。我们的病例为 MDA-5 皮肌炎的早期和积极治疗提供了证据，联合使用基于体重的泼尼松、MMF 和 IVIg。
