Myxovirus Resistance Protein 1-Expressing Fatal Myocarditis in a Patient with Anti-MDA5 Antibody-Positive Dermatomyositis.
Myxovirus Resistance Protein 1-Expressing Fatal Myocarditis in a Patient with Anti-MDA5 Antibody-Positive Dermatomyositis.
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DOI：
10.1093/rheumatology/keab637
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发表时间：
2021-08
期刊：
影响因子：
5.5
通讯作者：
Rika Sakamoto;Y. Kotobuki;Saki Iga;S. Nojima;R. Deno;Yuma Hanaoka;Kyoko Tonomura;E. Kiyohara;Y. Nakagawa;I. Ueda‐Hayakawa;N. Arase;M. Fujimoto
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文献类型：
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作者：
Rika Sakamoto;Y. Kotobuki;Saki Iga;S. Nojima;R. Deno;Yuma Hanaoka;Kyoko Tonomura;E. Kiyohara;Y. Nakagawa;I. Ueda‐Hayakawa;N. Arase;M. Fujimoto
亲爱的编辑，抗黑色素瘤分化相关基因5（抗-MDA 5）抗体（Ab）是一种肌炎特异性自身抗体，与临床上无肌病性DM伴间质性肺病（ILD）密切相关。虽然糖尿病相关的心肌炎很常见，但严重的心肌炎很少报告。我们报告一例抗MDA 5抗体阳性糖尿病与致命性心肌炎表达粘病毒耐药蛋白1（MxA）。一位48岁男性在入院前6个月开始出现皮疹、手指僵硬和MCP关节疼痛。随后出现眼睑水肿、近端肢体肌肉无力和疼痛以及用力时呼吸短促。入院时，观察到典型的DM皮肤表现，包括上眼睑的日光性皮疹;手指关节上的Gottron和手掌丘疹（图1A和B）;以及肩部、肘部（图1C）、手指关节、左阴囊和阴囊的多发性挖掘性皮肤溃疡，坏死组织达到sc组织。上下肢近端肌力下降。虽然血清肌酸激酶和醛缩酶值正常，但MRI显示上下肢近端肌肉信号广泛增加，提示肌病。血液检查显示抗MDA 5 Ab的血清阳性（5140指数，正常：< 32指数，通过ELISA测量）和AMA、PR 3-ANCA和MPO-ANCA的血清阴性。观察到血清铁蛋白（5380 ng/ml，正常：13- 277 ng/ml）、Krebs von den Lungen-6（KL-6; 2380 U/ml，正常：105- 401 U/ml）和脑钠肽（BNP; 373 pg/ml，正常：<40 pg/ml）水平升高。肩部红斑的皮肤活检显示界面皮炎，无血管炎。CT显示中度ILD。
DEAR EDITOR, Anti-melanoma differentiation–associated gene 5 (anti-MDA5) antibody (Ab) is a myositis-specific autoantibody closely associated with clinically amyopathic DM with interstitial lung disease (ILD). Although myocarditis associated with DM is common, severe myocarditis is rarely reported. We report a case of anti-MDA5 Ab–positive DM with fatal myocarditis expressing myxovirus-resistance protein 1 (MxA). A 48-year-old man started experiencing rashes, stiff fingers, and MCP joint pain 6months before admission to our department. Eyelid oedema, proximal limb muscle weakness and pain, and shortness of breath on exertion occurred subsequently. On admission, typical skin manifestations with DM were noted, including a heliotropic rash on the upper eyelids; Gottron’s and palmar papules on the finger joints (Fig. 1A and B); and multiple excavating skin ulcers with necrotic tissues reaching the sc tissues in the shoulders, elbows (Fig. 1C), finger joints, left buttock, and scrotum. The upper and lower limb proximal muscle strength decreased. Though the serum creatine kinase and aldolase values were normal, MRI revealed a widespread increase in the signals in the upper and lower limb proximal muscles, suggesting myopathy. Blood examination revealed seropositivity for anti-MDA5 Abs (5140 index, normal:< 32 index, measured by ELISA) and seronegativity for AMA, PR3-ANCA and MPO-ANCA. Increased serum levels of ferritin (5380ng/ml, normal: 13–277ng/ml), Krebs von den Lungen-6 (KL-6; 2380U/ml, normal: 105–401U/ml), and brain natriuretic peptide (BNP; 373pg/ml, normal:< 40pg/ml) were noted. Skin biopsy of the erythema on the shoulder showed interface dermatitis without vasculitis. CT revealed moderate ILD.