What is the management approach for a patient with chronic interstitial lung disease (ILD) characterized by coarse interstitial lung markings without acute findings?

Management of Chronic Interstitial Lung Disease with Coarse Interstitial Markings

For patients with chronic interstitial lung disease (ILD) characterized by coarse interstitial markings without acute findings, treatment should be tailored to the specific ILD subtype, with mycophenolate mofetil being the preferred first-line agent for most connective tissue disease-associated ILDs, while antifibrotic medications like nintedanib are recommended for progressive fibrosing ILDs. 1

Diagnostic Evaluation

Before initiating treatment, proper characterization of the ILD is essential:

• High-Resolution CT (HRCT): Gold standard for confirming ILD diagnosis with ~91% sensitivity and 71% specificity 2
• Pulmonary Function Tests (PFTs): To establish baseline and assess severity (restrictive pattern with reduced FVC, normal FEV1/FVC ratio, and reduced DLCO) 1
• Underlying cause assessment: Evaluate for connective tissue diseases, environmental exposures, and other potential etiologies

Treatment Algorithm Based on ILD Subtype

1. Connective Tissue Disease-Associated ILD (CTD-ILD)

For all CTD-ILD except systemic sclerosis:

• First-line: Glucocorticoids (prednisone) combined with immunosuppressive agents 3, 1
• Preferred immunosuppressant: Mycophenolate mofetil 1
• Alternative options:
  • Azathioprine
  • Rituximab
  • Cyclophosphamide 3

For Systemic Sclerosis-ILD (SSc-ILD):

• Strongly recommended against: Glucocorticoids as first-line treatment 3
• First-line options:
  • Mycophenolate mofetil
  • Nintedanib (conditionally recommended) 3
  • Tocilizumab (conditionally recommended) 3

For Inflammatory Myopathy-ILD (IIM-ILD):

• First-line options:
  • JAK inhibitors (conditionally recommended) 3
  • Calcineurin inhibitors (tacrolimus preferred over cyclosporine) 3

2. Progressive Fibrosing ILD (PF-ILD)

For patients showing disease progression (defined as decline in FVC ≥10% predicted, decline in FVC 5-10% with worsening symptoms or increased fibrosis on HRCT, or worsening symptoms with increased fibrosis) 1:

• Antifibrotic therapy:
  • Nintedanib: Slows FVC decline by 44-57% 1, 2
  • Pirfenidone: Consider for IPF but conditionally recommended against as first-line for SARD-ILD 3, 4

Monitoring and Follow-up

• Initial evaluation: Short-term PFTs (within 3 months) and HRCT (within 6 months) to determine progression rate 3, 1
• Regular monitoring:
  • Moderate-to-severe ILD: PFTs every 3-6 months
  • Mild ILD (FVC ≥70% and <20% fibrosis on HRCT): PFTs every 6 months for 1-2 years 3, 1
• Disease progression marker: A 5% decline in FVC over 12 months is associated with doubled mortality 1, 2

Supportive Care

• Oxygen therapy:

  • Long-Term Oxygen Therapy (LTOT) for patients with resting oxygen levels ≤7.3 kPa
  • Consider LTOT for oxygen levels ≤8 kPa if peripheral edema or pulmonary hypertension is present
  • Portable oxygen for patients with severe breathlessness 1

• Cough management:

  • For refractory cough, consider gabapentin and multimodality speech pathology therapy
  • In palliative settings, low-dose opiates may be considered for symptomatic relief when other treatments fail 3

• Preventive measures:

  • Pneumocystis jirovecii pneumonia prophylaxis with trimethoprim-sulfamethoxazole for patients on high-dose corticosteroids
  • Calcium and vitamin D supplementation
  • Vaccinations (influenza, pneumococcus, COVID-19) 1

Advanced Disease Management

• Early referral for lung transplantation should be considered for patients with progressive ILD despite optimal medical management 3, 1

• Pulmonary hypertension: Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. Consider inhaled treprostinil which can improve walking distance and respiratory symptoms 2, 5

Important Considerations

• A multidisciplinary approach involving pulmonologists, rheumatologists, radiologists, and pathologists is crucial for optimal management 3
• Treatment decisions should prioritize morbidity, mortality, and quality of life outcomes
• Early identification of progressive pulmonary fibrosis is essential for timely initiation of antifibrotic therapy 3
• Patients with coarse interstitial markings without acute findings should be monitored closely as this may represent early fibrotic changes that could progress over time