Idiopathic pulmonary fibrosis
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Idiopathic pulmonary fibrosis (IPF), or (formerly[5]) fibrosing alveolitis, is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function.[3][4] The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs.[6] Symptoms typically include gradual onset of shortness of breath and a dry cough.[1] Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails (nail clubbing).[1] Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism.[1]
Idiopathic pulmonary fibrosis | |
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Other names | Fibrosing alveolitis, cryptogenic fibrosing alveolitis, diffuse fibrosing alveolitis, usual interstitial pneumonitis, diffuse interstitial pneumonitis |
Figure A shows the location of the lungs and airways in the body. The inset image shows a detailed view of the lung's airways and air sacs in cross-section. Figure B shows fibrosis (scarring) in the lungs. The inset image shows a detailed view of the fibrosis and how it damages the airways and air sacs.[1] | |
Specialty | Pulmonology |
Symptoms | Shortness of breath, dry coughing[1] |
Complications | Pulmonary hypertension, heart failure, pneumonia, pulmonary embolism[1] |
Usual onset | Gradual[1] |
Causes | Unknown[2] |
Risk factors | Cigarette smoking, certain viral infections, family history[1] |
Diagnostic method | CT scan, lung biopsy[3] |
Differential diagnosis | Sarcoidosis, other interstitial lung diseases, hypersensitivity pneumonitis[4] |
Treatment | Pulmonary rehabilitation, supplemental oxygen, lung transplantation[1] |
Medication | Pirfenidone, nintedanib[2] |
Prognosis | Life expectancy ~ 4 years[1] |
Frequency | 12 per 100,000 people per year[4] |
The cause is unknown, hence the term idiopathic.[2] Risk factors include cigarette smoking, acid reflux disease (GERD), certain viral infections, and genetic predisposition.[1] The underlying mechanism involves scarring of the lungs.[1] Diagnosis requires ruling out other potential causes.[3] It may be supported by a HRCT scan or lung biopsy which show usual interstitial pneumonia (UIP).[3] It is a type of interstitial lung disease (ILD).[3]
People often benefit from pulmonary rehabilitation and supplemental oxygen.[1] Certain medications like pirfenidone (Esbriet) or nintedanib (Ofev) may slow the progression of the disease.[2] Lung transplantation may also be an option.[1]
About 5 million people are affected globally.[7] The disease newly occurs in about 12 per 100,000 people per year.[4] Those in their 60s and 70s are most commonly affected.[4] Males are affected more often than females.[4] Average life expectancy following diagnosis is about four years.[1] Updated international guidelines were published in 2022, which some simplification in diagnosis and the removal of antacids as a possible adjunct therapy.[8]