TABLE 2

Current recommendations for the pharmacological treatment of idiopathic pulmonary fibrosis (IPF) [36]

AgentGuidance#Strength of guidance
WarfarinStrong recommendation against useModerate confidence in effect estimates
NAC, prednisone and azathioprineStrong recommendation against useLow confidence in effect estimates
NAC monotherapyConditional recommendation against useLow confidence in effect estimates
BosentanConditional recommendation against useLow confidence in effect estimates
MacitentanConditional recommendation against useLow confidence in effect estimates
AmbrisentanStrong recommendation against useLow confidence in effect estimates
SildenafilConditional recommendation against useModerate confidence in effect estimates
ImatinibStrong recommendation against useModerate confidence in effect estimates
Anti-acid therapyConditional recommendation for useVery low confidence in effect estimates
NintedanibConditional recommendation for useModerate confidence in effect estimates
PirfenidoneConditional recommendation for useModerate confidence in effect estimates

NAC: N-acetylcysteine; #: Strong recommendation: most patients would want the suggested course of action. Conditional recommendation: the majority of patients would want the suggested course of action. Different choices will be appropriate for different patients depending on individual values and preferences. : Although anti-acid therapy received a conditional recommendation for use in this guideline, this was not based on evidence from prospective randomised controlled trials. No such trials have been conducted and are needed to determine the risk–benefit ratio in patients with IPF without symptomatic gastroesophageal reflux.