What is the treatment approach for an older patient with idiopathic pulmonary fibrosis (Interstitial Lung Disease, ILD) caused by asbestos exposure, considering a lung transplant?

Treatment of Asbestos-Related ILD with Lung Transplantation

Lung transplantation should be considered early for patients with asbestos-related ILD (asbestosis) who have severe or progressive disease, with referral recommended for patients under age 65 years who demonstrate functional impairment, oxygen dependency, or deteriorating course. 1

Immediate Transplant Evaluation Criteria

Early referral to a transplant center is critical because waiting times for donor organs can exceed 2 years, and patients with rapidly progressive fibrotic ILD may die while awaiting transplantation. 1

Specific Indications for Transplant Referral:

• Age <65 years (relative limit depending on local practice and comorbidities) 1
• DLCO <39% predicted 1
• FVC decline >10% over 6 months 1
• Severe functional impairment with oxygen dependency 1
• Progressive physiologic deterioration despite optimal medical management 1

Pre-Transplant Medical Management

While awaiting transplantation, supportive care is essential:

Oxygen Therapy

• Long-term oxygen therapy is recommended for patients with severe hypoxemia at rest (PaO₂ criteria for chronic respiratory failure) 1
• Monitor oxygen saturation at rest and with exertion at 3-6 month intervals 1
• Prescribe supplemental oxygen for desaturation below 88% during 6-minute walk test 1

Pulmonary Rehabilitation

• Respiratory rehabilitation programs should be initiated in patients with exercise limitation causing significant impairment 1
• May include exercise training, smoking cessation, psychosocial assistance, and supportive care 1
• May not be feasible in patients with advanced disease 1

Vaccinations

• Annual influenza vaccination is recommended 1
• Anti-pneumococcal vaccination using polysaccharide pneumococcal vaccine 1

Monitoring Protocol

• Monitor every 3-6 months with pulmonary function testing (FVC, DLCO) 1
• Assess for complications including pulmonary hypertension, pulmonary embolism, and cardiac dysfunction 1
• Evaluate oxygenation at rest and with exertion at each visit 1

Transplant Procedure Considerations

Single lung transplantation is currently the preferred surgical operation for fibrotic ILD including asbestosis. 1

The guideline committee did not make a definitive recommendation between single versus bilateral lung transplantation due to insufficient evidence, though bilateral transplantation is increasingly performed. 1 The decision must consider organ scarcity—bilateral transplantation uses organs that could potentially benefit two patients with single-lung procedures. 1

Contraindications to Transplantation

Relative Contraindications:

• Unstable or inadequate psychosocial profile/stability 1
• Significant extrapulmonary disorders (liver, renal, or cardiac dysfunction) that may negatively influence survival 1
• Age >60-65 years (varies by center) 1

Expected Outcomes

Post-Transplant Improvements:

• Arterial oxygen tension sufficiently improved to alleviate supplemental oxygen requirement 1
• Lung volumes and DLCO increased 1
• Pulmonary hypertension and right ventricular dysfunction reversed 1

Survival Data:

• 5-year survival approximates 50-60% after transplantation 1
• Early mortality causes: graft failure, infection, heart failure 1
• Late mortality causes: bronchiolitis obliterans, infection, malignancy 1

Critical Pitfalls to Avoid

Do not delay transplant referral until end-stage disease develops—patients should receive information about lung transplantation early in the disease course and undergo early assessment at a transplant center. 1 The median survival of untreated progressive fibrotic ILD is 2-5 years from diagnosis, making early listing essential. 2

Do not rely solely on antifibrotic therapy (pirfenidone, nintedanib) as these medications may slow progression but do not reverse fibrosis or eliminate the need for transplantation in severe disease. 3, 4

Ensure comprehensive comorbidity management before listing, particularly gastroesophageal reflux, pulmonary hypertension, coronary artery disease, and malignancy screening, as these significantly impact transplant candidacy and outcomes. 3