Johns Hopkins Hospital, 600 N Wolfe Street, Osler 292-A, Baltimore, MD 21287, USA.
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, 1830 East Monument Street, 5th Floor, Baltimore, MD 212015, USA.
Clin Chest Med. 2019 Sep;40(3):561-572. doi: 10.1016/j.ccm.2019.05.004.
The idiopathic inflammatory myopathies (IIMs), including polymyositis (PM) and dermatomyositis (DM), are autoimmune connective tissue diseases with variable degrees of muscle inflammation and systemic involvement. Interstitial lung disease (ILD) is a common complication of the IIMs and is associated with increased mortality. Many patients with PM/DM have myositis-specific and myositis-associated antibodies (MSA/MAAs) that result in distinct clinical phenotypes. Among these MSAs, anti-aminoacyl-tRNA antibodies and anti-melanoma differentiation factor 5 antibodies have high rates of ILD. Corticosteroids are the mainstay of treatment, although the addition of other immunosuppressive therapy is typically necessary to achieve disease control.
特发性炎性肌病（IIM），包括多发性肌炎（PM）和皮肌炎（DM），是具有不同程度肌肉炎症和全身受累的自身免疫性结缔组织疾病。间质性肺病（ILD）是 IIM 的常见并发症，与死亡率增加相关。许多 PM/DM 患者存在肌炎特异性和肌炎相关抗体（MSA/MAAs），导致不同的临床表型。在这些 MSA 中，抗氨酰-tRNA 抗体和抗黑色素瘤分化因子 5 抗体有很高的ILD 发生率。皮质类固醇是治疗的主要药物，尽管通常需要添加其他免疫抑制疗法来实现疾病控制。
