皮肌炎的肌炎特异性抗体:台湾33例个案的单中心研究

IF 4.6 Q2 MATERIALS SCIENCE, BIOMATERIALS ACS Applied Bio Materials Pub Date : 2023-01-01 DOI:10.4103/ds.ds-d-22-00122
Wei-Ting Liu, Chao-Chun Yang
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引用次数: 2

摘要

皮肌炎(DM)是一种以独特的皮肤表现和炎症性肌病为特征的全身自身免疫性疾病。随着肌炎特异性抗体(MSA)的发现,可以根据MSA类型对DM患者进行分类,特别是那些有较高危及生命并发症风险的患者。本研究旨在探讨台湾地区糖尿病患者msa的临床意义。共有33例DM患者接受了MSA测试,其中26例为典型DM, 7例为淀粉样病变DM。男性13例,女性20例,平均诊断年龄49.6岁。33例DM患者中有26例(78.8%)检测到MSA,其中MSA检测频率最高的是抗黑色素瘤分化相关蛋白5 (MDA5)(10/33, 30.3%),其次是抗转录中介因子-1γ (TIF-1γ)(8/33, 24.2%)。33例患者中有6例(18.2%)出现吞咽困难,抗tif -1γ(+)患者(5/ 8,62.5%)比抗tif -1γ(-)患者(1/ 25,4.0%)更频繁发生吞咽困难。15例(45.5%)患者出现间质性肺疾病,抗mda5(+)患者(7/10,70.0%)比抗mda5(-)患者(8/23,34.8%)发生频率更高。4例(12.1%)患者检测到恶性肿瘤,其中抗mi -2(+)、抗tif -1γ(+)、抗ars(+)和MSA(-)各1例。6例(18.2%)患者死亡,其中4例为抗mda5(+)。抗mda5和抗tif -1γ是两种最常检测到的msa。特异性msa的存在与某种表型相关,在评估DM时整合msa有助于准确的患者管理。
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Myositis-specific antibodies in dermatomyositis: A single-center experience of 33 cases in Taiwan
Dermatomyositis (DM) is a systemic autoimmune disease characterized by unique cutaneous manifestations and inflammatory myopathies. With the discovery of myositis-specific antibodies (MSAs), patients with DM, especially those with a higher risk of life-threatening complications, can be classified according to the MSA type. This retrospective study aimed to investigate the clinical significance of MSAs in patients with DM in Taiwan. A total of 33 patients with DM who underwent the MSA test, including 26 with classic DM and 7 with amyopathic DM, were included. There were 13 men and 20 women, with a mean age at diagnosis of 49.6 years. MSA was detected in 26 (78.8%) of 33 patients with DM. The most frequently detected MSA was anti-melanoma differentiation-associated protein 5 (MDA5) (10/33, 30.3%) followed by anti-transcription intermediary factor-1γ (TIF-1γ) (8/33, 24.2%). Dysphagia was present in 6 (18.2%) of the 33 patients and more frequently developed in patients with anti-TIF-1γ (+) (5/8, 62.5%) than those with anti-TIF-1γ (−) (1/25, 4.0%). Interstitial lung disease was noted in 15 patients (45.5%) and developed more frequently in patients with anti-MDA5 (+) (7/10, 70.0%) than those with anti-MDA5 (−) (8/23, 34.8%). Malignancies were detected in 4 (12.1%) patients, with one each of anti-Mi-2 (+), anti-TIF-1γ (+), anti-ARS (+), and MSA (−). Mortality occurred in 6 (18.2%) patients, of whom 4 were anti-MDA5 (+). Anti-MDA5 and anti-TIF-1γ were the two most commonly detected MSAs. The presence of specific MSAs is associated with a certain phenotype, and integrating MSAs while evaluating DM aids in accurate patient management.
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来源期刊
ACS Applied Bio Materials
ACS Applied Bio Materials Chemistry-Chemistry (all)
CiteScore
9.40
自引率
2.10%
发文量
464
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