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临床无肌病性皮肌炎合并间质性肺病,抗MDA5和抗PL12抗体双阳性。

Clinically amyopathic dermatomyositis with interstitial lung disease double-positive for anti-MDA5 and anti-PL12 antibodies.

作者信息

Hiramatsu Toshiya, Murano Moeko, Nakai Shogo, Murakami Yurina, Nishimoto Koji, Matsushima Sayomi, Harada Masanori, Uto Tomohiro, Sato Jun, Imokawa Shiro, Suda Takafumi

机构信息

Division of Respiratory Medicine, Iwata City Hospital, 512-3 Okubo, Iwata, Shizuoka, 438-8550, Japan.

Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, 431-3192, Japan.

出版信息

Respir Med Case Rep. 2022 Feb 18;36:101606. doi: 10.1016/j.rmcr.2022.101606. eCollection 2022.

Abstract

Anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl-tRNA synthetase (ARS) antibodies are two major myositis-specific autoantibodies with distinct clinical features. However, the clinical course remains unclear in patients with clinically amyopathic dermatomyositis (CADM)-interstitial lung disease (ILD) who have co-existing anti-MDA5 and anti-ARS antibodies. Here, we describe the case of a 32-year-old woman with CADM-ILD who had anti-MDA5 and anti-PL12 antibodies. Her serum ferritin level was within the normal range. However, chest computed tomography revealed bilateral lower-lobe consolidation and ground-glass opacities. Treatment with prednisolone and immunosuppressants was successful in improving the skin lesion and ILD, but relapse occurred on reducing the dose of prednisolone. These clinical features match those of anti-ARS antibody-positive dermatomyositis-ILD. Because these two conditions show significantly different clinical features and require different intensities of treatment, clinicians should carefully follow-up these patients throughout the course of the disease.

摘要

抗黑色素瘤分化相关基因5(MDA5)抗体和抗氨酰tRNA合成酶(ARS)抗体是两种具有不同临床特征的主要特发性炎性肌病自身抗体。然而,对于合并抗MDA5和抗ARS抗体的临床无肌病性皮肌炎(CADM)-间质性肺疾病(ILD)患者,其临床病程仍不明确。在此,我们描述了一名32岁患有CADM-ILD且有抗MDA5和抗PL12抗体的女性病例。她的血清铁蛋白水平在正常范围内。然而,胸部计算机断层扫描显示双侧下叶实变和磨玻璃影。泼尼松龙和免疫抑制剂治疗成功改善了皮肤病变和ILD,但在减少泼尼松龙剂量时病情复发。这些临床特征与抗ARS抗体阳性的皮肌炎-ILD相符。由于这两种情况表现出明显不同的临床特征且需要不同强度的治疗,临床医生在疾病全过程中应仔细随访这些患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a79/8866888/de044389c4f3/gr1.jpg

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