Quinn Caitlin, Wisse Amy, Manns Stephenie T
Emory Critical Care Center, 1364 Clifton Road, NE, Atlanta, GA 30322 USA.
2Medical University of South Carolina (MUSC), 25 Courtenay Drive, MSC 114, Charleston, SC 29425 USA.
Multidiscip Respir Med. 2019 Dec 2;14:35. doi: 10.1186/s40248-019-0197-0. eCollection 2019.
Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (ILD) with an unpredictable clinical course. Although IPF is rare, healthcare professionals should consider IPF as a potential cause of unexplained chronic dyspnea and/or cough in middle-aged/elderly patients and refer patients to a pulmonologist for evaluation. Making a diagnosis of IPF requires specialist expertise. Multidisciplinary discussion, involving at minimum a pulmonologist and a radiologist with expertise in the differential diagnosis of ILDs, is required to ensure the most accurate diagnosis. Prompt diagnosis of IPF is important to enable patients to receive appropriate care from an early stage. Optimal management of IPF involves the use of antifibrotic drugs, as well as the provision of supportive care to alleviate symptoms and preserve patients' quality of life. Antifibrotic drugs have been shown to slow lung function decline seen in patients with IPF. Patients' symptoms and functional capacity can be improved through participation in pulmonary rehabilitation programs and the use of supplemental oxygen. Patient education is essential to help patients understand and manage their disease. The identification and management of comorbidities, such as obstructive sleep apnea, pulmonary hypertension, and emphysema, is also an important element of the overall care of patients with IPF. Patients with IPF should be evaluated for lung transplantation at an early stage to maximize their chances of meeting eligibility criteria. In this review, we describe the clinical course and impact of IPF and best practice in its management, highlighting the importance of taking a patient-centered approach.
特发性肺纤维化（IPF）是一种进行性、致命的间质性肺疾病（ILD），临床病程不可预测。尽管IPF较为罕见，但医疗保健专业人员应将IPF视为中年/老年患者不明原因慢性呼吸困难和/或咳嗽的潜在病因，并将患者转诊至肺科医生处进行评估。诊断IPF需要专业知识。需要进行多学科讨论，至少包括一名肺科医生和一名在ILD鉴别诊断方面具有专业知识的放射科医生，以确保做出最准确的诊断。及时诊断IPF对于使患者能够从早期就接受适当治疗非常重要。IPF的最佳管理包括使用抗纤维化药物，以及提供支持性护理以缓解症状并维持患者的生活质量。抗纤维化药物已被证明可减缓IPF患者肺功能的下降。通过参与肺康复计划和使用补充氧气，可以改善患者的症状和功能能力。患者教育对于帮助患者了解和管理其疾病至关重要。识别和管理合并症，如阻塞性睡眠呼吸暂停、肺动脉高压和肺气肿，也是IPF患者整体护理的重要组成部分。IPF患者应尽早接受肺移植评估，以最大程度地提高符合资格标准的机会。在本综述中，我们描述了IPF的临床病程和影响及其管理的最佳实践，强调了采取以患者为中心方法的重要性。
