Idiopathic Pulmonary Fibrosis
Also known as: IPF, Cryptogenic Fibrosing Alveolitis
Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, and ultimately fatal disease characterized by the scarring (fibrosis) of the lung tissue. The cause is unknown (idiopathic), and the scarring leads to shortness of breath, cough, and reduced oxygen levels in the blood. The disease primarily affects older adults and has a variable rate of progression.
Imagine your lungs are like a sponge, and in IPF, the sponge gets hard and stiff, making it difficult to breathe. Doctors don't know exactly why this happens, but there are medicines to help slow it down.
Signs & Symptoms
- Shortness of breath (dyspnea), especially with exertion
- Chronic dry cough
- Fatigue
- Clubbing of the fingers and toes
- Weight loss
- Chest discomfort
Treatment Options
Pirfenidone
MODERATELY EFFECTIVE Approved 2014Nintedanib
MODERATELY EFFECTIVE Approved 2014Lung Transplantation
HIGHLY EFFECTIVEPulmonary Rehabilitation
SUPPORTIVEOxygen Therapy
SUPPORTIVEAntitussives
SUPPORTIVEDiagnosis
- High-resolution computed tomography (HRCT) scan of the chest
- Pulmonary function tests (PFTs)
- Lung biopsy (surgical or bronchoscopic)
- Bronchoalveolar lavage (BAL)
- Physical examination and medical history
History
IPF was first described in the mid-20th century, with increasing recognition and research into its pathogenesis and treatment options over the decades. The introduction of antifibrotic therapies in the 21st century marked a significant advancement in the management of the disease.
Recent Breakthroughs
TOPS Study: Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
The INBUILD trial demonstrated the efficacy of nintedanib in slowing disease progression in patients with progressive fibrosing interstitial lung diseases other than IPF.
Research on biomarkers for early detection of IPF
Ongoing research focuses on identifying biomarkers in blood and bronchoalveolar lavage fluid that can aid in early diagnosis and predict disease progression.