Setting Care Goals Early Is Key in Pulmonary Fibrosis

Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) and progressive pulmonary fibrosis (AE-PPF) continue to present complex challenges in intensive care settings, say researchers of a new paper, underscoring the high mortality associated with these events and the importance of early decision-making conversations and intervention strategies.1

IPF and PPF both face poor survival rates, and the pair share similar clinical features, leading to similar treatment approaches, which are limited and nuanced. | Image credit: Sebra - stock.adobe.com

Recent estimates suggest that AEs occur in up to 10% of patients with IPF and PPF annually. These episodes, marked by sudden respiratory decompensation and radiologic evidence of new ground-glass opacities on a background of usual interstitial pneumonia (UIP), are associated with dismal outcomes: in-hospital mortality reaches 50%, and rates approach 90% among those requiring mechanical ventilation.

IPF and PPF both face poor survival rates, and the pair share similar clinical features, leading to similar treatment approaches, which are limited and nuanced.

“Treatment options remain limited, often necessitating consideration for lung transplantation. Careful deliberation is required when deciding on intubation, and early integration of palliative care is strongly recommended from diagnosis through the disease course,” explained the researchers in Critical Care Nursing Clinics of North America.

Corticosteroids remain a cornerstone of AE-IPF and AE-PPF treatment following diagnosis, though their use is increasingly scrutinized based on questions surrounding their effectiveness and potential harm. While high-dose steroids may show some benefit in PPF flares, studies have failed to demonstrate survival benefit in AE-IPF, and concerns remain over immunosuppression risks. Retrospective studies comparing pulse-dose regimens (>250 mg/day of methylprednisolone) versus non-pulse doses (1 mg/kg/day) showed no significant outcome differences, also raising questions about optimal dosing strategies.

When patients get admitted to the intensive care unit for flares, delivery of oxygen varies based on specific needs of the patient. Options include high-flow nasal cannula (HFNC), preferred for its ability to provide enough oxygen and flow to help the patient breathe, as well as intubation and mechanical ventilation (MV).

Due to its association with high (90%) mortality, clinical guidelines hint at a recommendation against intubating patients, highlighting the need for early discussions with patients and their families to make decisions ahead of AEs. Researchers of the paper highlight that in cases of intubation, a careful approach should be taken to minimize any additional stress, such as ventilator-induced lung injury or ventilator-associated pneumonia, to the already compromised lungs.

Protective ventilation strategies include limiting plateau pressures (<30 cm H₂O), low tidal volumes (4-6 mL/kg), and optimizing positive end-expiratory pressure to reduce driving pressure.

Extracorporeal membrane oxygenation (ECMO), especially veno-venous ECMO (VV-ECMO), is emerging as a preferred alternative to MV in transplant-eligible patients, considered an evolved form of life support by acting as an artificial lung. ECMO supports gas exchange while minimizing lung injury and allowing physical therapy. Studies show favorable outcomes when ECMO is used as a bridge to transplant, with survival to discharge in more than half of patients. However, ECMO without a transplant plan confers limited benefit, with mortality as high as 75%.

Given the unpredictability and high mortality of acute exacerbations, early palliative care integration is essential in the disease states, explained the researchers. In a separate pilot study, researchers found that implementing an early palliative care program for patients with IPF significantly reduced the rate of deaths in a hospital setting based on patient preference. Notably, the study showed favorable survival among those who received early palliative care, though the study was limited to a single center and included a small group of 46 patients.2

While data have suggested benefits of integrating palliative care early on, referrals remain underutilized. One study showed that 92% of ICU survivors died within 2 months of discharge, emphasizing the need for clear goals-of-care discussions well before patients reach critical deterioration.

References

1. Ali A, Glassberg MK. Managing acute exacerbations in idiopathic pulmonary fibrosis and progressive pulmonary fibrosis in the intensive care unit setting. Crit Care Nurs Clin North Am. Published online July 4, 2025. doi:10.1016/j.cnc.2025.05.002

2. Bassi I, Pastorello S, Guerrieri A, et al. Early palliative care program in idiopathic pulmonary fibrosis patients favors at-home and hospice deaths, reduces unplanned medical visits, and prolongs survival: a pilot study. Eur J Intern Med. Published online May 23, 2024. doi:10.1016/j.ejim.2024.05.024