11th January 2024

Idiopathic pulmonary fibrosis

Fibrinolytics

• Pirfenidone and Nintedanib are the only two drugs approved for treatment of ILD in the UK.
• Pirfenidone has both anti-inflammatory and anti-fibrotic properties. It inhibits the production of transforming growth factor-β1 (TGF-β1), a cytokine involved in differentiation of human lung fibroblasts into myofibroblasts, thereby attenuating excess collagen and extracellular matrix deposition. Its anti-inflammatory properties come from its inhibition of several pro-inflammatory cytokines, including TNF-α, interleukin-1 (IL-1) and interferon-gamma (IFN-γ).
• Nintedanib is a tyrosine kinase inhibitor which inhibits multiple growth factors. Consequently, it prevents the proliferation and activation of fibroblasts and attenuates angiogenesis within the lungs. The result is a slowing down of fibrosis as well as associated complication of pulmonary hypertension.
• Both drugs were shown to slow down deterioration in FVC and DLCO when compared to non-treated cohort with IPF2. Patients with FVC of 50% to 80% are offered one of the medications. Treatment is stopped if FVC declines by >10% in 12 months while on the drug.
• They are generally well tolerated by patients; main side effect is gastrointestinal upset, which settles after a couple of months in most patients.
• Both medications are contraindicated in pregnancy and severe liver impairment.

Lung transplantation

• Patients with IPF should be referred for consideration of lung transplant soon after the diagnosis if there are no absolute contraindications and the patient is deemed to be fit enough for the procedure.