Possible preventive and therapeutic measures in acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF)
| Prevention | Recommendation |
| • 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 | + + +/− + +/− +/− −/+ + |
| Therapeutics | Recommendation |
| 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.