IPF

IPF stands for idiopathic pulmonary fibrosis, where idiopathic simply means the cause is unknown. It is the most common of the idiopathic interstitial pneumonias: a relentless scarring of the lungs that typically appears in older adults, more often in men and in current or former smokers. The word idiopathic is honest about a frustrating gap in understanding.

The disease is defined by a specific pattern of scarring called usual interstitial pneumonia, which can often be recognized on high-resolution CT by its subpleural, basal honeycombing and traction bronchiectasis. When the CT is characteristic, a tissue biopsy may not be needed; when it is uncertain, a multidisciplinary discussion and sometimes a surgical lung biopsy help confirm the diagnosis.

IPF tends to progress over time, though the speed varies and some people suffer abrupt declines called acute exacerbations. Symptoms are breathlessness, dry cough, fatigue, and finger clubbing. Two antifibrotic drugs, pirfenidone and nintedanib, have been shown to slow the decline in lung function, but they do not reverse existing scarring.

Because IPF is a diagnosis of a particular pattern with no identifiable cause, doctors must first carefully exclude mimics such as connective-tissue-disease ILD, chronic hypersensitivity pneumonitis, and drug or dust exposures. For suitable patients with advanced disease, lung transplantation remains the only treatment that can meaningfully extend survival.