Summary

• A rare form of fibrotic lung disease with no known aetiology that progresses over the course of several years and is characterised by scar tissue formation within the lungs, dyspnoea, and a significantly shortened lifespan after diagnosis.
• Although aetiology is unknown, cigarette smoking and certain environmental exposures have been implicated in the development of the disease.
• The diagnosis can be made on clinical grounds when an appropriate history of progressive symptoms (typically dyspnoea and cough) is accompanied by characteristic x-ray findings and restrictive pulmonary physiology, and in the absence of findings that suggest an alternative diagnosis.
• To date, no treatments have been shown to be effective in preventing or reversing the course of idiopathic pulmonary fibrosis. As a result, the most recent guidelines recommend against the use of pharmacological therapy in most patients, although some therapies are available for consideration in a minority of patients. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen when appropriate. Some patients may be eligible for lung transplantation.