Idiopathic pulmonary fibrosis medical therapy
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The mainstay of therapy for idiopathic pulmonary fibrosis is the supportive care measures which include mechanical ventilation, pulmonary rehbilitation, and vaccination against influenza and pneumococcus. Medical treatment as nintedanib and pirfenidone can be administrated to slow the disease progression.
- Patients with idiopathic pulmonary fibrosis receive supportive care alongside the medical therapy.
- Supportive care measures include the following:
- Mechanical ventilation: Nearly all the patients with IPF require oxygen supplementation.
- Pulmonary rehabilitation
- Vaccination against possible causes of pulmonary inflammation as influenza and pneumococcal polysacchride capsule vaccines.
- There is no specific treatment for idiopathic pulmonary fibrosis. However, there are two medications, nintedanib and pirfenidone, that can be administrated in order to slow the disease progression.
- Medical treatment may include the following therapies:
- Preferred regimen (1) : Nintedanib 150 mg PO q12h
- Preferred regimen (2):
- Day 1 to 3: Pirfenidone 267 mg PO q8h
- Days 8 to 14: Pirfenidone 534 mg PO q8h
- Follwoing days: Pirfenidone 801 mg PO q8h
- Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK; et al. (2011). "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management". Am J Respir Crit Care Med. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMC 5450933. PMID 21471066. Review in: Ann Intern Med. 2011 Jun 21;154(12):JC6-8
- Tomczyk S, Bennett NM, Stoecker C, Gierke R, Moore MR, Whitney CG; et al. (2014). "Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine among adults aged ≥65 years: recommendations of the Advisory Committee on Immunization Practices (ACIP)". MMWR Morb Mortal Wkly Rep. 63 (37): 822–5. PMC 5779453. PMID 25233284.
- Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J; et al. (2015). "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. 192 (2): e3–19. doi:10.1164/rccm.201506-1063ST. PMID 26177183.