idiopathic%20pulmonary%20fibrosis
IDIOPATHIC PULMONARY FIBROSIS

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. http://emedicine.medscape.com. Aug 2016.
  5. Godfrey AMK. Idiopathic pulmonary fibrosis workup. eMedicine. http://emedicine.medscape.com. Aug 2016.
  6. King TE, Jr. Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis. UptoDate. http://www.uptodate.com.. Aug 2016.
  7. King TE, Jr. Treatment of idiopathic pulmonary fibrosis. UptoDate. http://www.uptodate.com. Feb 2017.
  8. National Institute for Health and Care Excellence. Idiopathic pulmonary fibrosis in adults: diagnosis and management. NICE. https://www.nice.org.uk/. 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. http://www.uptodate.com. 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. http://www.uptodate.com. 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
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