Table 4.
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:
Moderate confidence in effect estimates.
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.