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.

Global incidence and prevalence of idiopathic pulmonary fibrosis

“…Idiopathic pulmonary fibrosis (IPF) is a rare chronic progressive disease of unknown etiology that affects both physical and emotional well-being [ 1 – 3 ]. It is characterized by irreversible loss of lung function due to fibrosis, which manifests as symptoms of increasing cough and dyspnea and impaired quality of life [ 2 – 6 ]. Lung transplantation is limited to a minority of patients and patients primarily rely on antifibrotic therapy plus several supportive/palliative treatments.…”

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.…”

Perioperative risk factors in patients with idiopathic pulmonary fibrosis: a historical cohort study

“…Nevertheless, diagnosis of IPF is complex and we did not tabulate multidisciplinary confirmation of IPF. 36 Secondly, we could not reliably stratify our study population based on the severity of IPF because of the complexities and controversies of ILD grading. 37 Although it has been shown that an absolute decline in FVC [ 10% or in DLCO [ 15% over a six-month time period is strongly associated with disease progression in non-surgical ILD patients, this study was inadequately powered to assess relationships between PFT values and postoperative outcomes.…”