The goals of treatment in IPF are essentially to reduce the symptoms, stop disease progression, prevent acute exacerbations and prolong survival. Preventive care (e.g. vaccinations) and symptom based treatment should be shared early in every patient.
A number of treatments have been investigated in the past for IPF, including interferon gamma-1β, bosentan, ambrisentan and anticoagulants, but there are no longer considered effective treatment options.
Many of these studies were based on the hypothesis that IPF is an inflammatory disorder.
Pirfenidone is a small molecule that combines both the anti-inflammatory, anti-oxidant, and anti-fibrotic effects in experimental models of fibrosis.
Pirfenidone marketed under the trade name ‘Esbriet’ is approved in Europe for the treatment of patients with mild-to-moderate IPF.
N-Acetylcysteine and Triple Therapy:
N-Acetylcysteine (NAC) is a precursor to glutathione, an antioxidant.
Treatment with high doses of NAC may repair an oxidant-antioxidant imbalance that occurs in the lung tissue of the patients with IPF.