From the Guidelines
Lung transplantation should be considered for patients with idiopathic pulmonary fibrosis (IPF) who experience progressive physiologic deterioration despite optimal medical management and meet established criteria, including severe functional impairment, oxygen dependency, and a deteriorating course 1. The criteria for lung transplant in patients with IPF include:
- Severe functional impairment
- Oxygen dependency
- A deteriorating course
- Patients should be under 60 years of age, although this may vary by center
- Patients should have no significant extrapulmonary disorders, such as liver, renal, or cardiac dysfunction, that may negatively influence survival
- Patients should have a stable and adequate psychosocial profile It is essential to note that the decision to list patients for transplantation is critical, as the 5-year survival after transplantation approximates 50 to 60% 1. According to the most recent guidelines, patients at increased risk of mortality should be referred for lung transplantation at diagnosis, and patients should be evaluated every 3–6 months or more often for disease progression 1. Early referral is crucial, as pulmonary fibrosis can progress rapidly, and the waiting time for procuring a suitable donor organ may exceed 2 years 1. The evaluation process involves comprehensive testing of cardiac, pulmonary, renal, and hepatic function, along with psychological assessment and infectious disease screening. Patients must complete pulmonary rehabilitation before transplant to optimize their physical condition. Transplantation offers the only definitive treatment for end-stage pulmonary fibrosis, with median survival post-transplant around 5-6 years, though outcomes continue to improve with advances in surgical techniques and immunosuppressive regimens.
From the Research
Criteria for Lung Transplant in IPF Patients
The criteria for lung transplant in patients with idiopathic pulmonary fibrosis (IPF) include several factors, such as:
- Age: Advanced age is a significant factor associated with failure to be listed for lung transplantation 2
- Percent predicted FVC: Lower FVC values are associated with increased likelihood of listing for lung transplantation 2
- Percent predicted DLCO: Lower DLCO values are associated with increased likelihood of listing for lung transplantation 2
- 6-min walk distance: Shorter 6-min walk distance is associated with increased likelihood of listing for lung transplantation 2
- Oxygen use at rest: Oxygen use at rest is a significant predictor of listing for lung transplantation 2
Disease Severity and Transplant Listing
Disease severity, as measured by FVC, DLCO, and dyspnea, does not appear to be directly associated with improvement in clinical outcomes after pulmonary rehabilitation 3. However, patients with advanced IPF who are referred for lung transplantation may still benefit from pulmonary rehabilitation, with improvements in exercise capacity and health-related quality of life 3.
Transplant Outcomes
Lung transplantation can improve survival and quality of life for selected patients with IPF 4, 5, 6. However, post-transplant survival is lower for IPF patients compared to other diseases, such as cystic fibrosis or chronic obstructive pulmonary disease, due to older age and associated comorbidities 5. The main cause of long-term mortality is late graft dysfunction, with other complications including infections and tumors 5.
Listing for Transplantation
Only a small proportion of potentially eligible patients with IPF are listed for lung transplantation, even when seen at pulmonary fibrosis centers of excellence 2. Early referral to a transplant unit for assessment is recommended for all patients diagnosed with IPF, without contraindications for surgery 5. The decision to list a patient for transplantation should be based on individual patient characteristics and the experience of each center 5.