Idiopathic pulmonary fibrosis
Rare, progressive lung disease with unknown cause and scarring.
Idiopathic pulmonary fibrosis (IPF), once called cryptogenic fibrosing alveolitis, is a rare and progressive respiratory disease. It causes the lung tissue around the air sacs to thicken and stiffen, leading to scar formation. This is a form of chronic pulmonary fibrosis that results in a steady, irreversible loss of lung function.
Symptoms usually develop gradually and include shortness of breath and a dry cough. Other signs can be fatigue and clubbing, where the fingertips or toenails become enlarged and dome-shaped. Possible complications include pulmonary hypertension, heart failure, pneumonia, or a pulmonary embolism.
The cause is unknown—hence the term "idiopathic." Known risk factors include cigarette smoking, gastroesophageal reflux disease, certain viral infections, and genetic predisposition. The disease process involves lung scarring. Diagnosis requires excluding other causes and can be supported by a high-resolution CT scan or lung biopsy showing usual interstitial pneumonia. IPF is a type of interstitial lung disease.
Treatment often helps: pulmonary rehabilitation and supplemental oxygen can improve quality of life. Anti-fibrotic drugs such as pirfenidone or nintedanib may slow disease progression. Lung transplantation is another option.
Globally, about 5 million people have IPF. Each year, roughly 12 new cases occur per 100,000 people. It most commonly affects people in their 60s and 70s, and men more often than women. Average life expectancy after diagnosis is about four years. Updated international guidelines from 2022 simplified the diagnostic process and removed antacids as a recommended adjunct therapy.
**Signs and symptoms**
Many people have symptoms for a considerable time before diagnosis. Common clinical features include being over 50, a dry cough that occurs with exertion, progressive shortness of breath during exercise, and fine crackling or popping sounds heard in the lungs during inhalation (especially at the bases of the lungs). Clubbing of the fingers or toes is also common. Pulmonary function tests typically show restriction and impaired gas exchange.
Some of these signs—like clubbing—result from chronic low oxygen levels in the blood and are not unique to IPF; they can occur in other lung diseases.
Quick Facts
- Field
- Pulmonology
- Symptoms
- Shortness of breath, dry coughing
- Complications
- Pulmonary hypertension, heart failure, pneumonia, pulmonary embolism
- Onset
- Gradual
- Causes
- Unknown
- Risks
- Cigarette smoking, certain viral infections, family history
- Diagnosis
- CT scan, lung biopsy
- Differential
- Sarcoidosis, other interstitial lung diseases, hypersensitivity pneumonitis
- Treatment
- Pulmonary rehabilitation, supplemental oxygen, lung transplantation
- Medication
- Pirfenidone, nintedanib
- Prognosis
- Life expectancy ~ 4 years
- Frequency
- 12 per 100,000 people per year
Facts from the source article.
Lore & Background
Idiopathic pulmonary fibrosis is a disease of unknown cause, hence the term idiopathic. Risk factors include cigarette smoking, gastroesophageal reflux disease, certain viral infections, and genetic predisposition. The underlying mechanism involves scarring of the lungs, believed to result from an aberrant wound healing process with abnormal deposition of collagen and minimal inflammation. Cellular senescence is suspected to be a central contributing cause. Diagnosis requires ruling out other potential causes and may be supported by high resolution CT scan or lung biopsy showing usual interstitial pneumonia. Symptoms include gradual onset of shortness of breath, dry cough, fatigue, and clubbing of digits. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism. Updated international guidelines published in 2022 simplified diagnosis and removed antacids as a possible adjunct therapy.
Reader's Guide
Idiopathic pulmonary fibrosis is significant as a progressive and ultimately fatal respiratory disease with a median survival of about four years after diagnosis. Its cause remains unknown despite extensive investigation, which drives ongoing research into aberrant wound healing pathways and genetic factors such as mutations in surfactant proteins and telomerase genes. The disease affects about 5 million people globally, with higher incidence in older adults and males. Diagnosis has been refined through multidisciplinary approaches and high-resolution CT imaging. Treatment options include pulmonary rehabilitation, supplemental oxygen, anti-fibrotic medications like pirfenidone or nintedanib that may slow progression, and lung transplantation. The 2022 guideline updates simplified diagnosis and removed antacids as therapy. The disease's legacy includes highlighting the need for early detection, as fine crackles on auscultation can raise suspicion, and for continued research into targeted therapies addressing fibroblast activation and extracellular matrix synthesis.
Did You Know?
- Idiopathic pulmonary fibrosis was formerly known as cryptogenic fibrosing alveolitis.
- Cigarette smoking increases the risk of IPF by about twofold.
- Familial IPF accounts for less than 5% of total patients and is clinically and histologically indistinguishable from sporadic IPF.
- The MUC5B genetic variant is found in approximately 20% of individuals with Northern and Western European ancestry.
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