Treatment

• Encourage measures to prevent exacerbations and slow disease progression:

  • Counsel on smoking cessation and treat tobacco-related disorders.

  • Ensure recommended vaccinations are administered.

  • Recommend avoidance of triggers for secondary causes of ILD.

• Offer pulmonary rehabilitation.

• Supplementary oxygen therapy as needed

• Symptom management

  • Consider cough suppressants and referral for multimodal speech therapy.

  • Consider palliative pharmacotherapy for dyspnea as indicated, e.g., palliative anxiolysis.

• Pharmacological therapy

  • Antifibrotic agents may reduce mortality and acute exacerbations and slow the decline in FVC.

    • Pirfenidone: inhibits TGF-β-stimulated collagen synthesis

    • Nintedanib: inhibits tyrosine kinases that target fibrogenic growth factors, e.g., VEGF, PDGF, and fibroblast growth factor receptor

  • Immunosuppressive therapy is not indicated.

• Lung transplantation: Refer for evaluation at the time of diagnosis.

• Pharmacological therapy: depending on the cause of ILD

  • Treat the underlying condition if possible, e.g.:

    • Specific management for patients with CTD-ILD

    • Antibiotics if bacterial interstitial pneumonia is suspected

  • Consider corticosteroids and/or systemic immunomodulators, e.g., for COP or NSIP, in consultation with a specialist.

• Lung transplantation

  • In patients with fibrotic NSIP, refer for evaluation at the time of diagnosis.

  • Refer patients with other types of non-IPF ILD and:

    • Progression to respiratory failure

    • ILD refractory to disease-specific therapies

Lung transplantation is the only curative option for ILD.

• Pulmonary hypertension

• Cor pulmonale

• Respiratory failure: initially partial respiratory failure (↓ pO2), followed by global respiratory failure (↑ pCO2) in advanced stage