Skip to main content
. 2017 Apr 3;13:427–437. doi: 10.2147/TCRM.S81141

Table 4.

ATS/ERS/JRS/ALAT recommendations for the treatment of IPF (2015 update)

Recommendation Therapy
Conditional for use Pirfenidonea
Nintedaniba
Antiacid therapyb
Conditional against use NAC monotherapyb
Phosphodiesterase-5 inhibition with sildenafila
Dual ERA antagonism with macitentanb
Dual ERA antagonism with ambrisentanb
Strong against use “Triple” therapy with azathioprine, NAC, and prednisoneb
Anticoagulation with warfarinb
Selective ERA antagonism with ambrisentanb
Imatiniba
Deferred Therapy for Class 3 PH associated with IPF
Single vs bilateral lung transplantation

Notes:

a

Moderate confidence in effect estimates.

b

Low confidence in effect estimates.

Abbreviations: ERA, endothelin receptor antagonist; IPF, idiopathic pulmonary fibrosis; NAC, N-acetylcysteine; PH, pulmonary hypertension; ATS/ERS/JRS/ALAT, American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association.