What is the treatment for interstitial fibrosis?

Treatment for Interstitial Fibrosis

The treatment for interstitial fibrosis should be tailored based on the underlying etiology, with antifibrotic medications such as nintedanib and pirfenidone as first-line options for idiopathic pulmonary fibrosis (IPF), while immunosuppressive therapies like mycophenolate are preferred for systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD). 1, 2

Treatment Based on Etiology

Idiopathic Pulmonary Fibrosis (IPF)

• First-line options:

  • Nintedanib: Strong evidence for slowing disease progression in IPF 2
  • Pirfenidone: Alternative first-line option at 2403 mg/day in divided doses 2, 3
  • Both medications have been shown to reduce the decline in forced vital capacity (FVC) 3

• Not recommended for IPF:

  • Combination therapy with prednisone, azathioprine, and N-acetylcysteine (strong recommendation against) 1
  • Imatinib 1
  • Ambrisentan 1

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

First-line treatment options:

• For all SARD-ILD except SSc-ILD:
  • Glucocorticoids (conditionally recommended) 1
• For all SARD-ILD:
  • Mycophenolate (preferred option) 1
  • Azathioprine 1
  • Rituximab 1
  • Cyclophosphamide 1
• For SSc-ILD and MCTD-ILD:
  • Tocilizumab 1
• For SSc-ILD:
  • Nintedanib (conditionally recommended) 1
  • Strongly recommended against glucocorticoids due to risk of scleroderma renal crisis 1
• For IIM-ILD:
  • JAK inhibitors 1
  • Calcineurin inhibitors 1

For progressive disease despite first-line treatment:

• Mycophenolate (if not used initially) 1
• Rituximab 1
• Cyclophosphamide 1
• Nintedanib (particularly for progressive fibrosing disease) 1
• For RA-ILD: Consider adding pirfenidone 1
• For SSc-ILD, MCTD-ILD, or RA-ILD: Consider tocilizumab 1
• For IIM-ILD: Consider JAK inhibitors, calcineurin inhibitors, or IVIG 1

Monitoring and Follow-up

• Regular monitoring with clinical visits and pulmonary function tests every 3-6 months 2
• High-resolution CT (HRCT) if there is concern for acute exacerbation, unexplained clinical changes, or suspected lung cancer 2
• Monitor for treatment side effects:
  • Pirfenidone: Gastrointestinal symptoms, photosensitivity, and skin rashes 4
  • Nintedanib: Primarily gastrointestinal side effects 4

Supportive Care and Symptom Management

• Pulmonary rehabilitation to improve exercise capacity and quality of life 2
• Oxygen therapy for patients with clinically significant resting hypoxemia or exercise desaturation 2
• Annual influenza and pneumococcal vaccinations 2
• Early integration of palliative care for symptom management 2
• Treatment of comorbidities such as gastroesophageal reflux disease (GERD) 2

Important Considerations and Pitfalls

1. Dose adjustments: Studies show that even reduced doses of antifibrotic medications may provide survival benefits compared to no treatment when full doses aren't tolerated due to side effects 5

2. Treatment adherence: The 801 mg tablet formulation of pirfenidone may improve adherence compared to the 267 mg formulation by reducing pill burden 6

3. Rapidly progressive ILD: Requires more aggressive approach, potentially including IV glucocorticoids (except in SSc), cyclophosphamide, rituximab, and consideration for early lung transplant referral 1

4. Combination therapy: For SARD-ILD receiving mycophenolate without evidence of progression, adding nintedanib or pirfenidone is conditionally not recommended 1

5. Clinical trials: Patients should be informed about available clinical trials at all stages of disease 2

By following these evidence-based treatment approaches and carefully monitoring disease progression and treatment response, the management of interstitial fibrosis can be optimized to slow disease progression and improve quality of life.