What are the current recommendations for managing interstitial lung diseases in the absence of the 2025 European Respiratory Society (ERS) guidelines?

Current Recommendations for Managing Interstitial Lung Diseases

The most current recommendations for managing interstitial lung diseases (ILDs) are based on the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guidelines, which provide comprehensive treatment approaches tailored to specific ILD subtypes. 1

Disease Classification and Diagnostic Approach

• High-Resolution CT (HRCT) is the gold standard for confirming ILD diagnosis (sensitivity ~91%, specificity 71%)
• Pulmonary Function Tests (PFTs) typically show a restrictive pattern with reduced DLCO
• Multidisciplinary discussion involving pulmonologists, rheumatologists, radiologists, and pathologists is essential for accurate diagnosis 2

First-Line Treatment Recommendations by ILD Subtype

Systemic Sclerosis-ILD (SSc-ILD)

• Strongly recommended against glucocorticoids as first-line treatment due to risk of scleroderma renal crisis 1
• Conditionally recommended first-line options (in order of preference):
  1. Mycophenolate mofetil
  2. Rituximab
  3. Nintedanib
  4. Tocilizumab (particularly in early progressive SSc-ILD with diffuse skin involvement)
  5. Cyclophosphamide

Inflammatory Myopathy-ILD (IIM-ILD)

• Conditionally recommended first-line options:
  1. Mycophenolate mofetil
  2. Rituximab
  3. Calcineurin inhibitors (tacrolimus preferred over cyclosporine)
  4. Nintedanib
  5. Cyclophosphamide
  6. JAK inhibitors (particularly for anti-MDA-5 positive cases) 1, 2

Mixed Connective Tissue Disease-ILD (MCTD-ILD)

• Conditionally recommended first-line options:
  1. Mycophenolate mofetil
  2. Rituximab
  3. Nintedanib
  4. Tocilizumab (particularly with SSc phenotype)
  5. Cyclophosphamide

Rheumatoid Arthritis-ILD (RA-ILD)

• Conditionally recommended first-line options:
  1. Mycophenolate mofetil
  2. Rituximab
  3. Nintedanib (particularly with UIP pattern)
  4. Tocilizumab
  5. Cyclophosphamide

Sjögren's Disease-ILD (SjD-ILD)

• Conditionally recommended first-line options:
  1. Mycophenolate mofetil
  2. Rituximab
  3. Nintedanib
  4. Cyclophosphamide

Management of Progressive ILD Despite First-Line Treatment

For patients with progression despite first-line therapy:

1. Assess adequacy of current treatment:

   • If suboptimal dosing due to intolerance, consider switching to alternative first-line therapy
   • If adequate dosing but progression continues, consider adding or switching therapy

2. Treatment options for progressive ILD (disease-specific recommendations):

   • SSc-ILD, MCTD-ILD, RA-ILD: Consider tocilizumab, nintedanib (particularly with UIP pattern), rituximab, or referral for stem cell transplantation/lung transplantation 1
   • RA-ILD: Consider adding pirfenidone (conditionally recommended) 1
   • IIM-ILD: Consider calcineurin inhibitors, JAK inhibitors, IVIG (particularly with respiratory muscle weakness) 1
   • All SARD-ILD: Plasma exchange is conditionally recommended against 1

Management of Rapidly Progressive ILD

For rapidly progressive ILD:

1. Pulse intravenous methylprednisolone (conditionally recommended as first-line) 1

2. Additional therapies (2 or more recommended in severe disease):

   • Rituximab
   • Cyclophosphamide
   • IVIG (particularly when infection is a concern)
   • Mycophenolate
   • Calcineurin inhibitors
   • JAK inhibitors

3. Consider early referral for lung transplantation 1, 2

Monitoring and Follow-up

• Short-term PFTs (within 3 months) and HRCT (within 6 months) to determine progression rate
• Regular monitoring with PFTs every 3-6 months for moderate-to-severe ILD and every 6 months for mild ILD
• A 5% decline in FVC over 12 months is associated with doubled mortality 2, 3

Supportive Care and Prevention of Complications

• Oxygen therapy for patients with hypoxemia (improves symptoms and quality of life) 2
• Structured exercise therapy (reduces symptoms and improves exercise capacity) 3
• Pneumocystis jirovecii pneumonia prophylaxis for patients on high-dose immunosuppression 2
• Vaccination (influenza, pneumococcus, COVID-19) 1
• Gastroesophageal reflux management with proton pump inhibitors or H2 blockers 1

Key Pitfalls to Avoid

1. Using glucocorticoids as first-line treatment in SSc-ILD - strongly recommended against due to risk of scleroderma renal crisis
2. Delaying treatment modification when progression is evident (defined as FVC decline ≥10%, or 5-10% with worsening symptoms)
3. Delaying referral for lung transplantation in patients with progressive disease
4. Failing to implement a multidisciplinary approach involving pulmonologists, rheumatologists, radiologists, and pathologists

While there are no 2025 ERS guidelines available yet, the 2023 ACR/CHEST guidelines represent the most current and comprehensive recommendations for managing ILDs in systemic autoimmune rheumatic diseases.