What are the treatment options for interstitial lung disease?

Treatment Options for Interstitial Lung Disease

Mycophenolate mofetil is the preferred first-line treatment for most types of interstitial lung disease (ILD), particularly those associated with systemic autoimmune rheumatic diseases (SARD-ILD), due to its favorable efficacy and safety profile. 1

First-Line Treatment Options by ILD Type

Systemic Autoimmune Rheumatic Disease-Associated ILD (SARD-ILD):

• Preferred first-line therapy: Mycophenolate mofetil 1
• Alternative first-line options:
  • Rituximab
  • Cyclophosphamide
  • Azathioprine

Disease-Specific Considerations:

1. Systemic Sclerosis-ILD:

   • Mycophenolate, rituximab, nintedanib, tocilizumab, cyclophosphamide 1
   • Strongly avoid glucocorticoids as first-line therapy (associated with scleroderma renal crisis) 1

2. Myositis-ILD:

   • Mycophenolate, rituximab, calcineurin inhibitors, nintedanib, cyclophosphamide 1

3. Rheumatoid Arthritis-ILD:

   • Mycophenolate, rituximab, nintedanib, tocilizumab, cyclophosphamide 1

4. Sjögren's-ILD:

   • Mycophenolate, rituximab, nintedanib, cyclophosphamide 1

5. Idiopathic Pulmonary Fibrosis (IPF):

   • Antifibrotic therapy with nintedanib or pirfenidone (slows FVC decline by 44-57%) 2, 3, 4

Management of Progressive ILD Despite First-Line Therapy

For patients who show progression despite first-line treatment:

1. Switch to alternative therapy or add-on therapy based on:

   • Current therapy
   • Disease type
   • Pattern on CT chest (UIP pattern vs. other)
   • Pace of progression 1

2. Preferred options:

   • Rituximab
   • Nintedanib (especially with fibrotic disease or UIP pattern)
   • Cyclophosphamide (not typically used in combination with other therapies)
   • Tocilizumab (particularly for SSc-ILD with early progressive disease, diffuse skin involvement, and elevated acute phase reactants) 1

3. Disease-specific second-line options:

   • Myositis-ILD: Consider calcineurin inhibitors or JAK inhibitors 1
   • Severe progressive ILD: Consider IVIG (especially with respiratory muscle weakness) 1

Management of Rapidly Progressive ILD (RP-ILD)

For patients with rapidly progressive ILD:

1. First-line treatment:

   • Pulse intravenous methylprednisolone (500-1000mg daily for 3 days) 1, 2
   • Plus 1-2 additional therapies:
     • Rituximab (preferred)
     • Cyclophosphamide (preferred)
     • IVIG (if high infection concern)
     • Mycophenolate
     • Calcineurin inhibitor
     • JAK inhibitor (particularly for anti-MDA-5 positive patients) 1

2. Consider early lung transplant referral for severe cases 1, 2

Monitoring and Follow-up

• Regular pulmonary function tests (PFTs) every 3-6 months 2
• HRCT when clinically indicated 2
• A 5% decline in FVC over 12 months is associated with doubled mortality 4
• Monitor for medication side effects, particularly with immunosuppressive therapy 2

Supportive Care

• Oxygen therapy for patients who desaturate below 88% on exertion 4
• Pulmonary rehabilitation to improve exercise capacity and quality of life 4
• Pneumocystis jirovecii pneumonia prophylaxis for patients on immunosuppression 2
• Calcium and vitamin D supplementation to prevent osteoporosis 2
• Vaccination (influenza, pneumococcus, COVID-19) 2

Special Considerations

• Glucocorticoids: Generally avoided in SSc-ILD due to risk of renal crisis, but may be necessary in rapidly progressive disease with close monitoring 1
• Lung transplantation: Should be considered for end-stage ILD with median survival improving from <2 years to 5.2-6.7 years post-transplant 4
• Pulmonary hypertension: Common in advanced fibrotic ILD (up to 85%); consider inhaled treprostinil which can improve walking distance and respiratory symptoms 4

The treatment of ILD requires careful consideration of the underlying cause, disease severity, progression rate, and patient factors. Early diagnosis and prompt initiation of appropriate therapy are crucial for improving outcomes and quality of life.