Anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis exhibit three clinical phenotypes with different prognoses
Anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis exhibit three clinical phenotypes with different prognoses
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DOI：
10.55563/clinexprheumatol/df2oc3
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发表时间：
2022-02-01
影响因子：
3.7
通讯作者：
Li, T.
中科院分区：
文献类型：
--
作者：
Yang, Q.;Lyu, K.;Li, T.
目的探讨抗黑色素瘤分化相关基因5抗体(DM-MDA5(+))阳性的皮肌炎(DM)患者的不同亚型，为临床个体化治疗提供依据。结果96例DM-MDA5(+)患者分为3个亚组，不同亚组患者的临床表现和预后具有高度的异质性。第1类患者为风湿病轻型组，预后良好。2组患者为青年典型DM组，预后良好。第3组为老年快速进展性间质性肺病(RPILD)组，预后不良。结论DM-MDA5(+)患者近期预后较差。在DM-MDA5(+)患者中发现了三种不同预后的临床表型。
ObjectiveWe aimed to identify different subtypes of dermatomyositis (DM) patients positive with anti-melanoma differentiation associated gene 5 antibody (DM-MDA5(+)) for customised treatments to improve the outcomes.MethodsAmong 96 DM-MDA5(+) patients, subgroups with similar phenotypes were delineated using hierarchical clustering analysis of the clinico-biological characteristics. Classification and regression trees were used to build a classification model and survival analysis was used to evaluate the prognoses of subgroups.ResultsThree subgroups were identified among 96 DM-MDA5(+) patients, and patients in different subgroups had highly heterogenic manifestations and outcomes. Cluster 1 patients were referred to as mild group of rheumatologic patterns with good prognosis. Cluster 2 patients were referred to as young typical DM group with good prognosis. Cluster 3 patients were referred to as elderly rapidly progressive interstitial lung disease (RPILD) group with poor prognosis. A predictive model to classify patients was established, and three critical factors were found, including age, serum ferritin and myalgia.ConclusionDM-MDA5(+)patients have a poor short-term prognosis. Three clinical phenotypes with different prognoses were identified in DM-MDA5(+) patients.