从细胞角度探讨炎性肌病相关间质性肺疾病的发病机制

Pathogenesis of idiopathic inflammatory myopathies-associated interstitial lung disease:cellular biology of the disease

特发性炎症性肌病(idiopathic inflammatory myopathies,IIM)是一种全身性异质性疾病,临床特征为骨骼肌炎症,伴或不伴有皮肤受累。间质性肺疾病(interstitial lung disease,ILD)是特发性炎症性肌病的严重并发症,也是其主要的致死原因之一,但其发病机制迄今仍不明确。本综述从细胞角度出发,梳理了相关细胞在IIM-ILD发病中可能起到的作用,并探讨了抗黑色素瘤分化相关蛋白5(melanoma differentiation-associated gene 5,MDA5)阳性皮肌炎与抗合成酶综合征继发性ILD之间的差异,为临床决策提供帮助。

Idiopathic inflammatory myopathy(IIM)is a systemic,heterogeneous disorder characterized clinically by inflammation of skeletal muscle with or without skin involvement.The most common lung involvement of IIM is interstitial lung disease(ILD).IIM-ILD has a negative impact on the prognosis of patients and the pathogenesis remains unclear to date.This review aims to sort out and compare the pathogenesis of IIM-ILD from different cellular perspectives.ILD incidence varies among subtypes of IIM patients.We explored the differences between anti-melanoma differentiation-associated gene 5(MDA5)-positive dermatomyositis and anti-synthetase syndrome in order to provide help for clinical decision.