Idiopathic pulmonary fibrosis

Video explanation

Idiopathic pulmonary fibrosis (IPF) is a chronic irreversible and fatal disease caused by a progressive decline in lung function.

The term pulmonary fibrosis means scarring of lung tissue and is the cause of worsening dyspnea (shortness of breath). Fibrosis is usually associated with a poor prognosis.

IPF belongs to a large group of more than 200 lung diseases known as interstitial lung diseases (ILDs), characterized by the involvement of lung interstitium.

IPF usually occurs in adults of between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women. Treatment to slow down the progression of the disease may include nintedanib or pirfenidone.

Idiopathic Pulmonary Fibrosis Media

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  Clubbing of the fingers in idiopathic pulmonary fibrosis

• Etiology and pathobiology of Idiopathic Pulmonary Fibrosis (IPF)

• Classification modifiée à la suite du Consensus international multidisciplinaire de l'American Thoracic Society et de l'European Respiratory Society

• A chest radiograph of a patient with IPF. Note the small lung fields and peripheral pattern of reticulonodular opacification.

• High-resolution computed tomography scans of the chest of a patient with IPF. The main features are of a peripheral, predominantly basal pattern of coarse reticulation with honeycombing

• Photomicrograph of the histopathological appearances of usual interstitial pneumonia. High-power magnification (on the right) shows a focus of fibroblastic proliferation, close to an area of fibrosis within which a mild, non-specific, chronic inflammatory cell infiltrate can be observed. In the subpleural space, a typical honeycombing aspect can be recognized.

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  Comparison of the 5-year survival rate for IPF and common malignancies. Adapted from Bjoraker et al. 1998.