多发性肌炎和皮肌炎中的间质性肺疾病。
Interstitial Lung Disease in Polymyositis and Dermatomyositis.
机构信息
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.
出版信息
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。
Zotero 插件