What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrosing interstitial pneumonia of unknown cause that occurs primarily in older adults. Its progression results in continuing fibrosis of the lung parenchyma and deterioration of lung function. Clinically, IPF most commonly presents with a slow onset of exertional dyspnea and dry cough.

Causes of Idiopathic Pulmonary Fibrosis

IPF is classified as an idiopathic interstitial pneumonia because its specific cause is unknown. However, research has identified some risk factors associated with its development:

- Smoking: There is a strong association between past or current smoking and IPF. Smoking damages the alveoli in the lungs over time which may increase the risk of fibrosis developing.

- Environmental exposures: Some cases of Idiopathic Pulmonary Fibrosis may be caused by repeated exposures to certain environmental pollutants, toxic gases or metals through occupations like metal work or farming.

- Genetics: Around 20% of IPF cases seem to have a genetic predisposition. Mutations in specific genes that control telomere length and repair have been linked to higher risk.

- Age: IPF most commonly affects people over 60 years of age. The risk increases with each decade of life.

- Gender: IPF is more prevalent in men than women by a ratio of roughly 2:1.

- Gastroesophageal reflux: Chronic acid reflux may contribute to inflammation and scarring in the lung tissue over time.

Pathogenesis of Idiopathic Pulmonary Fibrosis

The exact mechanisms that cause fibrosis or scarring of the lung tissue in Idiopathic Pulmonary Fibrosis are still unclear. However, current theories propose that repeated injury to the alveolar epithelial cells that line the lung triggers the activation and proliferation of fibroblasts in the lung. These fibroblasts go on to excessively deposit collagen proteins in the lung interstitium which gradually replaces the normal lung tissue and prevents effective gas exchange. Ongoing inflammation also likely plays a role in this pathological cycle.

Get More Insights On- Idiopathic Pulmonary Fibrosis

Unlock More Insights—Explore the Report in the Language You Prefer