TABLE 4

Possible preventive and therapeutic measures in acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF)

PreventionRecommendation
•Influenza and pneumococcal vaccination+
•Hand washing, avoidance of sick contacts+
•Approaches to minimise gastro-oesophageal reflex+/−
•Avoidance of airborne irritants and pollutants+
•When mechanical ventilation is required, strategies to minimise ventilator-induced lung injury+/−
  Low tidal volume ventilation
  Noninvasive ventilation
  High-flow nasal cannula oxygen therapy
•Nintedanib+/−
•Pirfenidone−/+
•Avoidance of the combination of prednisone and azathioprine+
TherapeuticsRecommendation
Ventilation
• Low tidal volume ventilation
• Noninvasive ventilation
• High-flow nasal cannula oxygen therapy
Pharmacology
• Corticosteroid
• Empiric antibiotics
• Immunosuppressant#
• Thrombomodulin
Lung transplantation
Others
• Extracorporeal membrane oxygenation
• Polymixin B haemoperfusion
• Rituximab, plasma exchange, intravenous immunoglobulin
• Non-steroid approach

+
+
+/−

+
+/−
−/+
−/+
+/−

§
−/+

+: Would consider using in most patients as potential benefit seems to outweigh potential harm; +/−: would consider using in selected patients as the balance of benefit and risk varies by clinical situation; −/+: would not consider using in majority of patients as the balance of benefit and risk varies by clinical situation; −: would not consider using in most patients as evidence is lacking to support a clinical benefit. #: Cyclophosphamide, cyclosporine, tacrolimus. : Immediate cessation of immunosuppression (if any), best supportive care, broad-spectrum antimicrobials. §: Would consider as a bridge to lung transplantation.