Idiopathic pulmonary fibrosis is the progressive parenchymal scarring & loss of pulmonary function due to unidentifiable cause of lung injury.

It is a specific form of chronic fibrosing interstitial pneumonia with the histopathologic characteristics of usual interstitial pneumonia.

Approximately 5% of patients are asymptomatic. Common signs and symptoms are exertional dyspnea, nonproductive cough, finger clubbing and bilateral inspiratory crackles.

  1. Betensley A, Sharif R, Karamichos D. A systematic review of the role of dysfunctional wound healing in the pathogenesis and treatment of idiopathic pulmonary fibrosis. J Clin Med. 2016 Dec;6(1):pii:E2. doi: 10.3390/jcm6010002. PMID: 28035951
  2. Bradley B, Branley HM, Egan JJ, et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008 Sep;63(Suppl 5):v1-58. doi: 10.1136/thx.2008.101691. PMID: 18757459
  3. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An International Working Group report. Am J Respir Crit Care Med. 2016 Aug;194(3):265-275. doi: 10.1164/rccm.201604-0801CI. PMID: 27299520
  4. Godfrey AMK. Idiopathic pulmonary fibrosis clinical presentation. eMedicine. Aug 2016.
  5. Godfrey AMK. Idiopathic pulmonary fibrosis workup. eMedicine. Aug 2016.
  6. King TE, Jr. Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis. UptoDate. Aug 2016.
  7. King TE, Jr. Treatment of idiopathic pulmonary fibrosis. UptoDate. Feb 2017.
  8. National Institute for Health and Care Excellence. Idiopathic pulmonary fibrosis in adults: diagnosis and management. NICE. May 2017.
  9. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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;183(6):788-824. doi: 10.1164/rccm.2009-040GL. PMID: 21471066
  10. Raghu G, Mikacenic C. Pathogenesis of pulmonary fibrosis. UptoDate. May 2016.
  11. Raghu G, Rochwerg B, Zhang Y, et al; American Thoracic Society; European Respiratory Society; Japanese Respiratory Society; Latin American Thoracic Association. 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;192(2):e3-19. doi: 10.1164/rccm.201506-1063ST. PMID: 26177183
  12. Schnapp LM, Whelan T. Acute exacerbations of idiopathic pulmonary fibrosis. UptoDate. Jan 2017.
  13. Tomika H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep. 2017 Jan;5(2):e00215. doi: 10.1002/rcr2.215. PMID: 28096998
  14. Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep;188(6):733-748. doi: 10.1164/rccm.201308-1483ST. PMID: 24032382
Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS Respirology - Malaysia digital copy today!
Sign In To Download
Editor's Recommendations
Most Read Articles
Roshini Claire Anthony, 11 Sep 2019

Beta-blockers could reduce mortality risk in patients with heart failure with reduced ejection fraction (HFrEF) and moderate or moderately-severe renal dysfunction without causing harm, according to the BB-META-HF* trial presented at ESC 2019.

Elvira Manzano, 2 days ago

The US Preventive Services Task Force (USPSTF), in an update of its 2013 recommendations, called on clinicians to offer risk-reducing medications to women who are at increased risk for breast cancer but at low risk for adverse effects.

Pearl Toh, 3 days ago
The use of SGLT-2* inhibitors was not associated with a higher risk of severe or nonsevere urinary tract infections (UTIs) in patients with type 2 diabetes (T2D) compared with DPP**-4 inhibitors or GLP-1*** receptor agonists, a population-based cohort study shows.
6 days ago
In type 2 diabetes patients taking sulfonylureas, hypoglycaemia duration is longer at night and is inversely correlated with the level of glycated haemoglobin (HbA1c), a new study reports.