Table 1– Evidence-based treatment recommendations
Strong yes
 Implication for the patient: most patients would want the following treatment intervention and only a small proportion would not.
  1) Long-term oxygen therapy in patients with IPF demonstrating clinically significant resting hypoxaemia
  2) Lung transplantation in appropriate patients
Weak yes
 Implication for the patient: a majority of patients would want the following treatment intervention, but many would not. Not using them may be a reasonable choice in a minority.
  1) Corticosteroids for acute exacerbation of IPF
  2) Treatment of asymptomatic gastro-oesophageal reflux
  3) Pulmonary rehabilitation
Strong no
 Implication for the patient: most patients would not want the following treatment intervention and only a small proportion would.
  1) Monotherapy with corticosteroids
  2) Colchicine
  3) Cyclosporine A
  4) Combined corticosteroid and immune modulator therapy
  5) Interferon-γ
  6) Bosentan
  7) Etanercept
Weak no
 Implication for the patient: the majority of patients would not want the following treatment intervention, but many would, i.e. the following treatment interventions should not be used in the majority of patients with IPF, but may be a reasonable choice in a minority.
  1) Combined prednisone, azathioprine and NAC
  2) Monotherapy with NAC
  3) Anticoagulation
  4) Pirfenidone
  5) Pulmonary hypertension associated with IPF
  6) Mechanical ventilation in patients with respiratory failure due to IPF
  • “Yes” is “for” the use of the specific treatment; “No” is “against” the use of the specific treatment. IPF: idiopathic pulmonary fibrosis; NAC: N-acetyl-cysteine. Data from 2.