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ATS/ERS/JRS/ALAT Clinical Practice Guideline: treatment of idiopathic pulmonary fibrosis: overview


Idiopathic pulmonary fibrosis ( IPF ) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause occurring in adults. Radiologic and/or histopathologic patterns are consistent with usual interstitial pneumonia.

The first guideline on the management of idiopathic pulmonary fibrosis was published in 2000 and was based on the consensus opinion of a group of international experts.
The next guideline was published in 2011 and was a rigorous, evidence-based, joint effort by the American Thoracic Society ( ATS ), European Respiratory Society ( ERS ), Japanese Respiratory Society ( JRS ), and Latin American Thoracic Association ( ALAT ).

The new guideline updates several recommendations for treatment from the previous guideline and provides new recommendations on topics not considered in the previous guideline.

1. The recommendation against the use of the following agents for the treatment of idiopathic pulmonary fibrosis is strong:

a. Anticoagulation ( Warfarin ) ( ++⊝⊝, low confidence in effect estimates ); b. Imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor ( PDGF ) receptors ( +++⊝, moderate confidence in effect estimates ); c. Combination prednisone, azathioprine, and N-acetylcysteine ( ++⊝⊝, low confidence in effect estimates ); d. Selective endothelin receptor antagonist ( Ambrisentan ) ( ++⊝⊝, low confidence in effect estimates ).

2. The recommendation for the use of the following agents for the treatment of idiopathic pulmonary fibrosis is conditional:

a. Nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors ( +++⊝, moderate confidence in effect estimates ); b. Pirfenidone ( +++⊝, moderate confidence in effect estimates ).

3. The recommendation against the use of the following agents for the treatment of idiopathic pulmonary fibrosis is conditional:

a.Phosphodiesterase-5 inhibitor ( Sildenafil ) ( +++⊝, moderate confidence in effect estimates ); b. Dual endothelin receptor antagonists ( Macitentan, Bosentan ) ( ++⊝⊝, low confidence in effect estimates ).

The following recommendations are unchanged from the 2011 guideline:

1. Updated evidence syntheses related to N-acetylcysteine monotherapy and antiacid therapy were presented to the panel, and both recommendations were left unchanged from the 2011 guideline ( a conditional recommendation against N-acetylcysteine monotherapy based on low confidence in effect estimate and a conditional recommendation for antiacid therapy based on very low confidence in effect estimate ).

2. An updated evidence synthesis related to the treatment of pulmonary hypertension associated with idiopathic pulmonary fibrosis was also presented to the panel, but decisions regarding modifying the recommendation from the 2011 guideline were deferred until the next update.

3. Recommendations for multiple other interventions that were addressed in the 2011 guideline ( e.g., treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroids, oxygen supplementation, mechanical ventilation, pulmonary rehabilitation, and lung transplantation in general ) were not prioritized for an update in this guideline,

An evidence synthesis was also performed for a new question about single versus bilateral lung transplantation, but decisions regarding a recommendation were deferred until the next version of the guideline to gather additional information that was felt necessary before formulating a recommendation.

Questions regarding newer treatments ( e.g., antibiotics ) were not addressed and were deferred until the next version of the guideline because of resource constraints. ( Xagena )

Source: American Thoracic Society, 2015

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