From the Guidelines
Idiopathic Pulmonary Fibrosis (IPF) is a chronic, fibrosing interstitial pneumonia of unknown cause that is associated with radiological and histologic features of usual interstitial pneumonia (UIP), primarily affecting older adults and characterized by progressive worsening of dyspnea and lung function, with a poor prognosis. This condition is marked by the scarring of lung tissue, which leads to thickening and stiffening of the lungs, making it difficult for oxygen to pass into the bloodstream 1. The key characteristics of IPF include:
- Chronic and progressive nature
- Unknown cause
- Association with usual interstitial pneumonia (UIP)
- Primary affectation of older adults
- Progressive worsening of dyspnea and lung function
- Poor prognosis As noted in the guidelines published in the American Journal of Respiratory and Critical Care Medicine, IPF diagnosis and management are crucial for improving patient outcomes 1. Treatment options and management strategies for IPF aim to slow disease progression, manage symptoms, and improve quality of life, and may include medications, oxygen therapy, pulmonary rehabilitation, and in severe cases, lung transplantation 1.
From the FDA Drug Label
What is pirfenidone Pirfenidone is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF).
Idiopathic Pulmonary Fibrosis (IPF) is a lung disease.
- The provided drug labels do not give a detailed description of IPF, they only mention that pirfenidone is used to treat IPF. 2
From the Research
Definition and Characteristics of Idiopathic Pulmonary Fibrosis (IPF)
- Idiopathic pulmonary fibrosis (IPF) is a progressive and devastating lung disease characterized by fibrosis and worsening lung function, primarily occurring in individuals 50 years and older 3.
- IPF is associated with symptoms such as cough, dyspnea, and impaired quality of life 4, 5.
- The disease leads to the destruction of lung parenchyma and architecture, resulting in lost compliance and compromised gas exchange 5.
Pathogenesis and Diagnosis of IPF
- The current concept of IPF pathogenesis involves repetitive injury to the lung epithelium, leading to fibroblast accumulation, myofibroblast activation, and deposition of matrix 4.
- Diagnosis requires a high index of clinical suspicion, supported by distinct radiographic and/or histopathologic findings, and often involves a multidisciplinary team-based discussion 4, 3.
- High-resolution computed tomography typically shows interstitial pneumonia with basal and peripheral honeycombing, and gas exchange and diffusion capacity are impaired 6.
Treatment and Management of IPF
- Current treatment options for IPF are limited, but antifibrotic drugs such as pirfenidone and nintedanib can slow disease progression and improve quality of life 4, 3, 5, 6.
- Lung transplantation is the only treatment shown to increase life expectancy in IPF patients, but it is often contraindicated due to age and comorbidities 5, 6.
- Pulmonary rehabilitation remains a critical part of IPF management, improving quality of life and functional level 3.