Clinical course and management of idiopathic pulmonary fibrosis

Abstract

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.

Differences in Baseline Characteristics and Access to Treatment of Newly Diagnosed Patients With IPF in the EMPIRE Countries

“…Healthcare systems deal differently with diagnostic possibilities and availability. Considering treatment, expensive therapies are often introduced later as in wealthier countries and might be limited to a selected population of IPF ( 14 , 15 ). Many off-label treatments are applied in rare diseases with potentially serious side effects ( 16 ).…”

Nutrition implications of intrinsic restrictive lung disease

“…Therefore, RDNs can address nutrition issues while providing credible, evidence‐based recommendations to patients with ILD. Previous research has identified patient education, symptom relief, and management of comorbidities as vital components of supportive care in ILD management 90 . RDNs can support patients in each of these three supportive care components using their nutrition expertise and skills.…”

“…Adverse events are the most common reason for patients to discontinue medications 89 . In general, medication‐related gastrointestinal (GI) adverse events can be managed through dose adjustment, treatment interruption, and/or symptoms management 90 . Therefore, involvement of healthcare professionals to provide symptomatic and supportive care to patients in order to manage these medication‐related adverse events is an important component of ILD/IPF management.…”

Critical Care Management of Lung Fibrosis, Review Article

“…Evidence throughout the literature provided by Quinn, Wisse, & Manns, (2019) [17], also highlights the efficacy and significance of pulmonary rehabilitation in patients suffering from shortness of breath due to pulmonary fibrosis and associated complications. It has been found that pulmonary rehabilitation is considered to be one of the prime critical care management strategies in case of permanent fibrosis as it provides significant knowledge and information to the patients regarding their health condition as well as the procedures they need to maintain to restore their health [18].…”