What Is the Treatment of Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease. First-line management follows a structured approach that integrates pharmacological and nonpharmacological strategies, with treatment decisions informed by the patient's clinical profile and risk.

Treatment Approach

Management combines antifibrotic pharmacological therapy with nonpharmacological interventions — the full protocol specifies the options available and the clinical criteria that guide their selection and use.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1164/rccm.202206-1098ST

Treatment considerations should include both pharmacological (nintedanib and pirfenidone) and nonpharmacological (oxygen supplementation and/or pulmonary rehabilitation) therapies.
Patients may benefit from involvement of palliative care to help with symptom management (cough, dyspnea, and/or anxiety).
Patients at increased risk of mortality should be referred for lung transplantation at diagnosis.

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