Living With Idiopathic Pulmonary Fibrosis by Ben's Friends

LivingwithIdiopathicPulmonaryFibrosis.org

Pirfenidone (Esbriet) and Nintedanib (Ofev), for treatment of IPF

What Are Pirfenidone (Esbriet) and Nintedanib (Ofev)?

In clinical trials, pirfenidone (Esbriet) and nintedanib (Ofev) have been shown to mitigate fibrosis or scarring, and therefore slow progression of the disease.

Pirfenidone has multiple possible mechanisms of action and is an oral medication. In Phase 3 clinical trials, nearly half the participants with mild to moderate idiopathic pulmonary fibrosis receiving pirfenidone saw a reduced decline in forced vital capacity. This measure indicates delayed progression of the disease. Participants experienced other positive benefits including improvements in 6-minute walking distance and decreases in mortality (number of deaths) during the 52-week study period. Possible adverse effects of treatment were manageable and limited to photosensitivity, stomach cramps, nausea and vomiting.

Nintenanib is tyrosine-kinase inhibitor and like pirfenidone is an oral medication. Among other biologic processes, tyrosine kinase is involved in scarring and fibrotic change. In Phase 3 clinical trials, like pirfenidone, nintenanib also slowed the progression of idiopathic pulmonary fibrosis as measured by a reduction in the rate of decline of forced vital capacity. Potential adverse effects include diarrhea, nausea, vomiting, headache, increased blood pressure, and increased liver enzymes (an indicator of liver dysfunction). Finally, Ofev is a teratogen that shouldn’t be taken during pregnancy for fear of fetal deformity or death.

What Do Pirfenidone (Esbriet) and Nintedanib (Ofev) Mean to You?

If you or someone you know has idiopathic pulmonary fibrosis, the approval of pirfenidone (Esbriet) and nintedanib (Ofev) Is a real medical breakthrough that should be welcomed as good news. However, some issues need to be clarified before these drugs truly find their place in clinical medicine. First, it’s unclear whether pirfenidone and nintendanib should be used separately or simultaneously when treating idiopathic pulmonary fibrosis. If used together, we’re unsure which drug should be used first. If used separately, we’re unsure whether the medications will display a synergistic or augmented effect. Second, we’re unsure whether some people are particularly responsive (“responders”) to either or both of these medications. Third, we don’t know if the drugs will continue to mitigate progression of idiopathic pulmonary fibrosis past the nearly yearlong test period.

https://www.verywellhealth.com/idiopathic-pulmonary-fibrosis-treatments-1123900