抗MDA5阳性的特发性炎性肌病的临床特征

Clinical characteristics of idiopathic inflammatory myopathy patients with positive anti-MDA5 antibody

目的肌炎特异性自身抗体(MSA)与特发性炎性肌病(IIM)的临床特征的关系尚未完全明确,本研究探讨MSA与IIM临床表现的关系。方法回顾62例进行了完整的MSA抗体检测的IIM患者。分析不同亚型IIM的临床表现、MSA检测结果以及MSA与临床表现的关系。结果皮肌炎(DM)和抗合成酶综合征容易合并间质性肺病(ILD),DM皮疹、关节炎发生率较高,而坏死性肌炎的肌无力、肌酸激酶升高和心肌损害比较显著。抗MDA5阳性患者ILD、快速进展性ILD、关节病变、溃疡性皮疹等表现也更常见,血抗SSA/Ro52抗体阳性比例、血清铁蛋白显著升高,肌酸激酶水平、外周血淋巴细胞计数显著降低。结论 DM患者特别是抗MDA5阳性的患者皮疹、关节炎和ILD发生率高,溃疡性皮疹、关节炎、高铁蛋白血症和外周血淋巴细胞减少有助于识别抗MDA5阳性的IIM亚型。

Objective To investigate the correlation between myositis-specific autoantibody(MSA) and the clinical characteristics of idiopathic inflammatory myopathy(IIM). Methods A total of 62 hospitalized IIM patients who received complete MSA antibody testing were enrolled in this study. The clinical manifestations of different IIM subtypes, MSA typing and their correlation were analyzed. Results Patients with dermatomyositis(DM) and anti-synthetase syndrome tended to develop interstitial lung disease(ILD). DM patients had a higher incidence of skin rashes and arthritis. Necrotizing myositis patients had significant higher prevalence of myasthenia, elevated creatine kinase and myocardial damage. Furthermore, ILD, rapidly progressive ILD, joint lesions and ulcerative skin rash were more common in patients with anti-MDA5 antibody. The anti-SSA/Ro52 antibody positive rate and serum ferritin level were significantly increased while the creatine kinase and peripheral blood lymphocyte count were significantly reduced in patients with positive anti-MDA5 antibody. Conclusion The incidence of rashes, arthritis, and ILD are high in DM patients, especially in those with positive anti-MDA5 antibody. Ulcerative rashes, arthritis, hyperferritinemia, and lymphocytopenia are clues for diagnosis of IIM patients with positive anti-MDA5 antibody.