From the Guidelines
Anti-fibrotic agents are medications that slow or prevent the development of fibrosis, with nintedanib and pirfenidone being the most established treatments, primarily used for idiopathic pulmonary fibrosis, and dosed at 150 mg twice daily and 801 mg three times daily, respectively. These medications work by targeting various pathways involved in fibrosis development, including inflammation, extracellular matrix production, and fibroblast activation 1. The treatment of systemic sclerosis with anti-fibrotic agents has been evaluated in several studies, with nintedanib showing a potential benefit on lung function, slowing the decline in FVC in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) 1. Pirfenidone has also been evaluated in SSc-ILD, with some studies suggesting it is safe and well-tolerated, although the evidence is less extensive compared to nintedanib 1.
Some key points to consider when using anti-fibrotic agents include:
- Nintedanib is approved in many countries for the treatment of SSc-ILD, whereas pirfenidone is not, although it may be considered in some cases 1
- The dose of nintedanib may need to be reduced to 100 mg twice daily if not tolerated at the full dose of 150 mg twice daily 1
- Pirfenidone should be increased to 801 mg three times daily as tolerated, with a reduced dose if not tolerated 1
- Both medications can cause side effects, with nintedanib frequently causing diarrhea and pirfenidone commonly causing gastrointestinal issues and photosensitivity 1
The use of anti-fibrotic agents in clinical practice should be guided by the most recent and highest-quality evidence, with a focus on improving morbidity, mortality, and quality of life for patients with fibrotic diseases 1. As such, nintedanib and pirfenidone should be considered as first-line treatments for patients with idiopathic pulmonary fibrosis and SSc-ILD, respectively, with the goal of slowing disease progression and improving quality of life.
From the FDA Drug Label
Pirfenidone is a pyridone indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF).
Anti-fibrotic agents are drugs that prevent or reduce the formation of excess fibrous connective tissue.
- Pirfenidone is an example of an anti-fibrotic agent used in the treatment of idiopathic pulmonary fibrosis (IPF) 2 2.
From the Research
Definition and Examples of Anti-Fibrotic Agents
- Anti-fibrotic agents are medications used to treat fibrotic diseases, such as idiopathic pulmonary fibrosis (IPF) 3, 4, 5.
- Examples of anti-fibrotic agents include pirfenidone and nintedanib, which have been shown to slow the decline in lung function in patients with IPF 3, 4, 5, 6, 7.
Mechanism of Action and Efficacy
- Pirfenidone and nintedanib have been found to reduce the rate of decline in forced vital capacity (FVC) among patients with IPF over time 3, 4, 5, 6, 7.
- These medications have also been shown to reduce the risk of acute respiratory deteriorations, which are associated with high morbidity and mortality 7.
Safety and Tolerability
- Pirfenidone and nintedanib have been found to have side effects, including gastrointestinal symptoms, photosensitivity, and skin rashes 4, 6.
- The incidence of adverse events with pirfenidone is lower than that with nintedanib, but both are generally well-tolerated 6.
- Dose adjustment has been shown to be effective at reducing side effects without compromising efficacy 7.
Clinical Use and Recommendations
- Pirfenidone and nintedanib are recommended for the treatment of patients with IPF, and have been shown to improve life expectancy 3, 4, 5, 6, 7.
- Prompt treatment with anti-fibrotic agents is critical to preserving patients' lung function and improving outcomes 7.
- A holistic approach to care, including symptom management and supportive care, is also important for individuals with IPF 7.