Therapeutic Approach to Adult Fibrotic Lung Diseases

被引:42
作者
Adegunsoye, Ayodeji [1 ]
Strek, Mary E. [1 ]
机构
[1] Univ Chicago, Dept Med, Sect Pulm & Crit Care, 5841 S Maryland Ave, Chicago, IL 60637 USA
基金
美国国家卫生研究院;
关键词
connective tissue disease; hypersensitivity pneumonia; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; interstitial lung disease; IDIOPATHIC PULMONARY-FIBROSIS; CHRONIC HYPERSENSITIVITY PNEUMONITIS; OBSTRUCTIVE SLEEP-APNEA; MESENCHYMAL STEM-CELLS; TYROSINE KINASE INHIBITOR; PLACEBO-CONTROLLED TRIAL; SCLERODERMA LUNG; DOUBLE-BLIND; GASTROESOPHAGEAL-REFLUX; INTERSTITIAL PNEUMONIA;
D O I
10.1016/j.chest.2016.07.027
中图分类号
R4 [临床医学];
学科分类号
100218 [急诊医学];
摘要
Among the interstitial lung diseases (ILDs), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis, and fibrotic connective tissue disease-related ILD are associated with a worse prognosis, with death occurring as a result of both respiratory failure and serious associated comorbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF. Although chronic hypersensitivity pneumonitis and connective tissue disease related-ILD may be associated with an inflammatory component, the evidence for the use of immunosuppressive agents in their treatment is largely limited to retrospective studies. The lack of benefit of immunosuppressive therapy in advanced fibrosis argues for rigorous clinical trials using antifibrotic therapies in these types of ILD as well. Patients with fibrotic ILD may benefit from identification and management of associated comorbid conditions such as pulmonary hypertension, gastroesophageal reflux, and OSA, which may improve the quality of life and, in some cases, survival in affected individuals. Because early assessment may optimize posttransplantation outcomes, lung transplant evaluation should occur early in patients with IPF and those with other forms of fibrotic ILD.
引用
收藏
页码:1371 / 1386
页数:16
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