What are the management approaches for Interstitial Lung Disease (ILD) according to the European Respiratory Society (ERS) ILD 2025 guidelines?

Management Approaches for Interstitial Lung Disease (ILD) According to ERS ILD 2025 Guidelines

The European Respiratory Society (ERS) ILD 2025 guidelines recommend mycophenolate mofetil as the preferred first-line treatment for most types of interstitial lung disease, particularly those associated with systemic autoimmune rheumatic diseases (SARD-ILD). 1

Disease Classification and First-Line Treatment Options

Systemic Sclerosis-ILD (SSc-ILD)

• First-line treatments:
  • Mycophenolate mofetil (preferred option) 2, 1
  • Nintedanib (conditionally recommended) 2, 1
• Strongly avoid glucocorticoids due to risk of scleroderma renal crisis 2, 1

Rheumatoid Arthritis-ILD (RA-ILD)

• First-line treatments:
  • Mycophenolate mofetil (preferred option) 1
  • Rituximab (especially with active inflammatory arthritis) 2, 1
  • Tocilizumab 1
• Note: Panel was unable to reach consensus on nintedanib as first-line therapy 2

Idiopathic Inflammatory Myopathy-ILD (IIM-ILD)

• First-line treatments:
  • Mycophenolate mofetil 1
  • JAK inhibitors (conditionally recommended) 2, 1
  • Calcineurin inhibitors (conditionally recommended) 2, 1

Other SARD-ILD (Sjögren's, MCTD, etc.)

• First-line treatments:
  • Mycophenolate mofetil (preferred option) 1
  • Rituximab 1
  • Cyclophosphamide 1

Management of Progressive Disease

For Progressive SSc-ILD

• Add nintedanib 2, 1
• Consider tocilizumab 2, 1
• Consider referral for stem cell transplantation or lung transplantation 2, 1

For Progressive RA-ILD

• Add pirfenidone 2, 1
• Consider tocilizumab 2, 1

For Progressive IIM-ILD

• Add calcineurin inhibitors 2
• Consider IVIG 2

Management of Rapidly Progressive ILD (RP-ILD)

First-line approach for RP-ILD

• Pulse intravenous glucocorticoids (except in SSc-ILD) 2, 1
• Combination therapy rather than monotherapy (except in SSc-ILD) 2
• Options include:
  • Rituximab 2, 1
  • Cyclophosphamide 2, 1
  • IVIG (especially for IIM-ILD and MCTD-ILD) 2, 1
  • Mycophenolate 2, 1
  • Calcineurin inhibitors 2, 1
  • JAK inhibitors 2, 1

For MDA-5 RP-ILD

• Triple combination therapy over monotherapy 2
• Consider adding IVIG to other therapies 2

Monitoring and Follow-up

• Pulmonary Function Tests (PFTs):

  • Every 3-6 months in moderate-to-severe ILD or progressive disease 2
  • A 5% decline in FVC over 12 months is associated with approximately 2-fold increase in mortality 3

• High-Resolution CT (HRCT):

  • Repeat within first 3 years after diagnosis 2
  • Repeat within 3-6 months to 1 year to determine treatment response 2

• Multidisciplinary Approach:

  • Regular collaboration between pulmonologists, rheumatologists, radiologists, and pathologists 2, 1
  • Virtual multidisciplinary team meetings to discuss complex cases 2

Treatment Contraindications and Cautions

• Avoid in SARD-ILD:

  • Pirfenidone as first-line treatment (conditionally recommended against) 2
  • Methotrexate, leflunomide, TNF inhibitors, abatacept 2, 1
  • Inhaled corticosteroids 1
  • IVIG or plasma exchange as first-line options 2

• Glucocorticoid Use:

  • Short-term use only (≤3 months) for most SARD-ILD 1
  • Strongly avoid in SSc-ILD due to risk of scleroderma renal crisis 2, 1
  • Avoid adding glucocorticoids for progressive disease 2

Advanced Disease Management

• Consider early referral for lung transplantation:

  • Especially in rapidly progressive disease 2
  • Pre-transplantation evaluation takes time and should be initiated early 2
  • Median survival after lung transplant is 5.2-6.7 years compared to <2 years without transplant in advanced ILD 3

• Stem cell transplantation:

  • Consider for progressive SSc-ILD 2
  • Generally not recommended as first-line treatment 2

Common Pitfalls to Avoid

1. Delayed treatment initiation: Early intervention is crucial to prevent irreversible fibrosis 2, 1

2. Inadequate monitoring: Regular PFTs and symptom assessment are essential to detect progression 2, 3

3. Inappropriate glucocorticoid use: Particularly dangerous in SSc-ILD 2, 1

4. Neglecting comorbidities: Conditions like GERD and pulmonary hypertension can exacerbate ILD 1

5. Late referral for transplantation: Early evaluation is critical for patients with progressive disease 2

By following these evidence-based management approaches, clinicians can optimize outcomes for patients with various forms of interstitial lung disease, focusing on early intervention, appropriate medication selection, and regular monitoring to prevent disease progression.