Miller Shelly A, Glassberg Marilyn K, Ascherman Dana P
Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine, University of Miami Miller School of Medicine, 1600 NW 10th Avenue, #1140, Miami, FL 33136, USA.
Division of Rheumatology, University of Miami Miller School of Medicine, Rosenstiel Medical Science Building 7152, 1600 Northwest 10th Avenue, Miami, FL 33136-1050, USA.
Rheum Dis Clin North Am. 2015 May;41(2):249-62. doi: 10.1016/j.rdc.2014.12.006. Epub 2015 Feb 27.
Pulmonary complications cause significant morbidity and mortality in the idiopathic inflammatory myopathies. Advances in biomarker discovery have facilitated clinical phenotyping, allowing investigators to better define at-risk patient subsets and to potentially gauge disease activity. This serologic characterization has complemented more traditional assessment tools. Pharmacologic management continues to rely on the use of corticosteroids, often in combination with additional immunosuppressive agents. The rarity of myositis-associated interstitial lung disease and lack of controlled trials have limited analyses of treatment efficacy, mandating the development of standardized outcome measures and improvement of data sharing between disciplines.
肺部并发症在特发性炎性肌病中可导致显著的发病率和死亡率。生物标志物发现方面的进展促进了临床表型分析,使研究人员能够更好地界定高危患者亚组,并有可能评估疾病活动度。这种血清学特征补充了更为传统的评估工具。药物治疗仍依赖于使用皮质类固醇,通常还会联合其他免疫抑制剂。肌炎相关间质性肺疾病的罕见性以及缺乏对照试验限制了对治疗疗效的分析,因此需要制定标准化的结局指标并改善各学科之间的数据共享。