IPF

Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive lung disease characterized by the thickening and scarring (fibrosis) of lung tissue. This condition leads to a gradual decline in lung function and is classified under the broader category of interstitial lung disease (ILD).

Pathophysiology

The exact cause of IPF is unknown, which is why it is termed "idiopathic." The disease involves the abnormal and excessive deposition of extracellular matrix proteins, leading to the stiffening of the lung tissue. This fibrosis impairs the ability of the lungs to transfer oxygen into the bloodstream, resulting in symptoms such as dyspnea (shortness of breath) and a persistent, dry cough.

Symptoms

Common symptoms of IPF include:

• Shortness of breath, especially during or after physical activity
• A dry, persistent cough
• Fatigue and weakness
• Unexplained weight loss
• Clubbing of the fingers or toes

Diagnosis

Diagnosing IPF typically involves a combination of clinical evaluation, imaging studies, and sometimes lung biopsy. Key diagnostic tools include:

• High-resolution computed tomography (HRCT) scans
• Pulmonary function tests (PFTs)
• Bronchoscopy with bronchoalveolar lavage (BAL)
• Surgical lung biopsy

Treatment

There is no cure for IPF, but treatments aim to manage symptoms and slow disease progression. Treatment options include:

• Antifibrotic medications such as pirfenidone and nintedanib
• Oxygen therapy
• Pulmonary rehabilitation
• Lung transplantation in severe cases

Prognosis

The prognosis for IPF varies, but the disease is generally progressive and can lead to respiratory failure. The median survival time after diagnosis is approximately 3-5 years.

Research

Ongoing research is focused on understanding the underlying mechanisms of IPF and developing new treatments. Clinical trials are continually being conducted to explore potential therapies.

Related Pages

• Interstitial lung disease
• Pulmonary fibrosis
• Dyspnea
• Pirfenidone
• Nintedanib
• Lung transplantation

See Also

• Respiratory failure
• Pulmonary rehabilitation
• Oxygen therapy