What is the treatment approach for interstitial lung disease (ILD)?

Treatment Approach for Interstitial Lung Disease (ILD)

For patients with interstitial lung disease (ILD), treatment should be tailored to the specific ILD subtype, with mycophenolate being the preferred first-line therapy for most systemic autoimmune rheumatic disease (SARD)-associated ILDs. 1

Treatment Based on ILD Subtype

Systemic Autoimmune Rheumatic Disease (SARD)-Associated ILD

First-line Treatment Options:

• Systemic Sclerosis (SSc)-ILD:

  • Mycophenolate (preferred) 1
  • Tocilizumab 1
  • Nintedanib (conditionally recommended) 1
  • AVOID: Glucocorticoids (strongly recommended against) 1

• Inflammatory Myopathies (IIM)-ILD:

  • Mycophenolate (preferred) 1
  • Azathioprine 1
  • JAK inhibitors 1
  • Calcineurin inhibitors 1
  • Short-term glucocorticoids 1

• Mixed Connective Tissue Disease (MCTD)-ILD:

  • Mycophenolate (preferred) 1
  • Azathioprine 1
  • Tocilizumab 1
  • Short-term glucocorticoids (use cautiously in patients with SSc phenotype) 1

• Rheumatoid Arthritis (RA)-ILD:

  • Mycophenolate (preferred) 1
  • Azathioprine 1
  • Cyclophosphamide 1
  • Short-term glucocorticoids 1

• Sjögren's Disease (SjD)-ILD:

  • Mycophenolate (preferred) 1
  • Azathioprine 1
  • Cyclophosphamide 1
  • Short-term glucocorticoids 1

Treatments NOT Recommended as First-line for SARD-ILD:

• Leflunomide
• Methotrexate
• TNF inhibitors
• Abatacept
• IVIG or plasma exchange 1

For Progressive ILD Despite First-line Treatment

1. For all SARD-ILD progression:

   • Mycophenolate
   • Rituximab
   • Cyclophosphamide
   • Nintedanib 1

2. For specific subtypes:

   • RA-ILD: Consider adding pirfenidone 1
   • SSc-ILD, MCTD-ILD, RA-ILD: Consider tocilizumab 1
   • IIM-ILD: Consider calcineurin inhibitors, JAK inhibitors 1
   • IIM-ILD and MCTD-ILD: Consider adding IVIG 1
   • SSc-ILD: Consider referral for stem cell transplantation and/or lung transplantation 1

Rapidly Progressive (RP)-ILD Management

For patients with SARD and rapidly progressive ILD:

• Pulse intravenous methylprednisolone as first-line treatment 1
• Consider rituximab, cyclophosphamide, IVIG, mycophenolate, calcineurin inhibitors, and JAK inhibitors 1
• For confirmed or suspected MDA-5, use triple therapy; for others, use double or triple therapy 1
• Early referral for lung transplantation is recommended 1

Idiopathic Pulmonary Fibrosis (IPF)

• Antifibrotic therapy with nintedanib or pirfenidone is recommended 2, 3
• Pirfenidone has been shown to reduce decline in FVC compared to placebo 2
• These medications slow annual FVC decline by approximately 44% to 57% 3

Monitoring and Supportive Care

Monitoring Disease Progression

• Regular pulmonary function tests (PFTs) - a 5% decline in FVC over 12 months is associated with approximately 2-fold increase in mortality 3
• Exercise capacity assessment (6-minute walk test)
• Symptom assessment including cough and dyspnea 1
• High-resolution CT scans 4

Supportive Care

• Structured exercise therapy to reduce symptoms and improve 6-minute walk test distance 3
• Oxygen therapy for patients who desaturate below 88% on a 6-minute walk test 3
• For ILD-associated cough, treat underlying ILD and investigate other potential causes (GERD, asthma, upper airway cough syndrome) 1

Special Considerations

• Pulmonary hypertension: Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. Consider inhaled treprostinil which improves walking distance and respiratory symptoms 3
• Lung transplantation: Should be considered for patients with end-stage ILD. After transplant, patients have a median survival of 5.2 to 6.7 years compared with less than 2 years without transplant 3

Common Pitfalls to Avoid

1. Using glucocorticoids in SSc-ILD - strongly recommended against due to risk of renal crisis 1
2. Delaying referral for lung transplantation - early referral is recommended for rapidly progressive ILD 1
3. Focusing only on lung-specific treatments - comprehensive approach addressing comorbidities and extrapulmonary manifestations is essential 5
4. Inadequate monitoring - regular assessment of pulmonary function, symptoms, and disease progression is crucial 4
5. Using treatments not recommended for specific ILD subtypes - treatment must be tailored to the specific ILD subtype 1

By following this structured approach based on ILD subtype and monitoring disease progression carefully, outcomes related to morbidity, mortality, and quality of life can be optimized.