Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a rare fibrosing interstitial pneumonia of unknown cause. The disease is chronic, progressive, and ultimately fatal.

Diagnosis is suspected clinically in patients with bi-basilar inspiratory crackles, older age (e.g., >60 years), and unexplained symptomatic or asymptomatic bilateral pulmonary fibrosis on a chest x-ray or high-resolution computed tomography.

In the absence of findings suggesting an alternative disease process, IPF is usually confirmed by multi-disciplinary review based on the presence of a radiological (high-resolution chest CT) pattern of usual interstitial pneumonia (UIP). Lung biopsy histopathology may be considered when radiological and clinical data result in an uncertain diagnosis.

Initial therapy is generally with an antifibrotic agent (either pirfenidone or nintedanib), although some patients might benefit from other treatment options depending on disease severity. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen.

Palliative care options and referral for lung transplantation should be considered early, even if a patient is being actively treated.

IPF is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown aetiology. It is defined by a radiological and/or histopathological pattern of UIP.[1]Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-68. https://www.atsjournals.org/doi/full/10.1164/rccm.201807-1255ST http://www.ncbi.nlm.nih.gov/pubmed/30168753?tool=bestpractice.com [2]Liu GY, Budinger GRS, Dematte JE. Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. BMJ. 2022 Jun 29;377:e066354. https://www.bmj.com/content/377/bmj-2021-066354.long http://www.ncbi.nlm.nih.gov/pubmed/36946547?tool=bestpractice.com ​ 

The disease is limited to the lungs, so presentation should occur without constitutional or other symptoms that suggest a multisystem disease or an alternative diagnosis.[1]Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-68. https://www.atsjournals.org/doi/full/10.1164/rccm.201807-1255ST http://www.ncbi.nlm.nih.gov/pubmed/30168753?tool=bestpractice.com

Diagnosis requires the exclusion of other forms of interstitial pneumonia, including other idiopathic interstitial pneumonias and interstitial lung diseases associated with environmental exposures, medications, or systemic diseases (e.g., connective tissue disease).[3]Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824. https://www.atsjournals.org/doi/full/10.1164/rccm.2009-040GL http://www.ncbi.nlm.nih.gov/pubmed/21471066?tool=bestpractice.com [4]Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19. https://www.atsjournals.org/doi/full/10.1164/rccm.201506-1063ST http://www.ncbi.nlm.nih.gov/pubmed/26177183?tool=bestpractice.com [5]Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47. https://www.atsjournals.org/doi/10.1164/rccm.202202-0399ST http://www.ncbi.nlm.nih.gov/pubmed/35486072?tool=bestpractice.com