Acute Exacerbations of IPF, PFF, Require Careful Clinical Decisions

Patients with acute exacerbations of idiopathic pulmonary fibrosis (IPF) or progressive pulmonary fibrosis (PPF) face a high risk of morbidity and mortality and require careful, expert management, according to a new report.1

Given the high risk, the goals of treatment should be discussed with patients early on, the authors explained. The study was published in Critical Care Nursing Clinics of North America.

Both IPF and PPF are subsets of interstitial lung disease (ILD), but there are important differences that should inform treatment.

IPF and PPF are subsets of interstitial lung disease with important differences that should inform treatment. | Image credit: PRASANNAPIX - stock.adobe.com

Patients diagnosed with IPF should be quickly referred to a lung transplant or ILD center, since lung transplantation remains the only curative treatment for the disease. However, since most patients will be deemed ineligible for lung transplant, the investigators said the primary focus of therapy should be to prevent or slow further decline. That can involve the use of the antibiotic agents pirfenidone (Esbriet; Genentech) and nintedanib (Ofev; Boehringer Ingelheim). Still, while such medications can lower the risk of acute exacerbations, the mean survival for patients with IPF remains short, at just 1 to 3 years.

Survival among PPF is somewhat better than that of IPF, the authors noted, with one study finding the 4-year survival rate of people with PPF to be 62%.2 Treatment for PPF typically is based on the underlying pathophysiology, the authors said, including immunosuppression for patients whose disease is tied to autoimmune causes. Nintedanib can be used to slow the decline of forced vital capacity, they said. However, pirfenidone is not recommended for PPF since the authors said the data supporting its use in PFF has been inconclusive.

Patients who experience flares of IPF or PPF typically present with acute to subacute worsening of dyspnea over 30 days, the investigators said. It is important for clinicians to ensure there are no other causes of worsening dyspnea, such as pulmonary embolism and ischemic heart disease, they noted. In these patients, computed tomography of the chest during an exacerbation will show new ground-glass opacities over a usual interstitial pneumonia pattern, they said.

Steroids are one option for treatment, though the data supporting the use of steroids is stronger for PPF than for IPF. That may be due to patients with IPF being sicker, they noted.

Mechanical ventilation offers something of a paradox in IPF and PPF, according to the authors. While it can be a life-saving intervention, patients who require mechanical ventilation have a much higher mortality rate. They noted that one study found patients with IPF needing mechanical ventilation had a 56% mortality rate, compared to a 7.5% mortality rate among patients who did not require the therapy.3 Furthermore, mechanical ventilation is generally regarded as a contraindication to transplant, the authors said.

“The decision to intubate a patient with IPF and PPF requires careful consideration of several factors, particularly the likelihood of survival and the availability of a potential bridge to transplant,” they wrote.

Another option for patients eligible for transplant is extracorporeal membrane oxygenation (ECMO), which they said can act as a bridge to recovery or transplant. However, they said patients ineligible for transplant may not benefit from ECMO. Therefore, it is important that clinicians have ongoing discussions with patients about the goals of care.

“Given the difficulty in predicting the clinical course in these patients, palliative care consultation or involvement should start at the time of diagnosis,” they wrote.

They said palliative care is “imperative” in cases of acute exacerbation of IPF or PPF, but it remains an underutilized resource.

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. 2025;37(3):407-419. doi:10.1016/j.cnc.2025.05.002

2. Chen T, Zeng C. Compare three diagnostic criteria of progressive pulmonary fibrosis. J Thorac Dis. 2024;16(2):1034-1043. doi:10.21037/jtd-23-481

3. Mooney JJ, Raimundo K, Chang E, Broder MS. Mechanical ventilation in idiopathic pulmonary fibrosis: a nationwide analysis of ventilator use, outcomes, and resource burden. BMC Pulm Med. 2017;17(1):84. doi:10.1186/s12890-017-0426-2