From the Guidelines
Treatment for possible lung fibrosis should prioritize antifibrotic therapy, such as nintedanib 150mg twice a day, to slow disease progression and improve quality of life. According to the most recent and highest quality study 1, nintedanib has been shown to be effective in slowing the decline in lung function in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).
Treatment Options
- Antifibrotic therapy: Nintedanib 150mg twice a day is recommended, with the option to reduce the dose to 100mg twice a day if not tolerated 1.
- Immunosuppression: Oral mycophenolate mofetil (MMF) or azathioprine may be considered, with MMF showing potential benefits in slowing lung function decline when used in combination with nintedanib 1.
- Rigorous anti-reflux therapy and treatment of dysphagia: Proton pump inhibitors, H2 antagonists, prokinetic drugs, and lifestyle modifications are recommended to manage gastroesophageal reflux and dysphagia 1.
- Other interventions: Smoking cessation, vaccinations, oxygen therapy, exercise programs, and pulmonary rehabilitation are also important components of treatment 1.
Symptom Management
- Cough suppressants and treatment of gastroesophageal reflux with proton pump inhibitors may be used to manage symptoms.
- Vaccination against respiratory infections is essential to prevent exacerbations.
Disease Monitoring
- Regular evaluation of disease progression, including lung function tests and high-resolution computed tomography (HRCT) scans, is crucial to adjust treatment plans accordingly 1.
By prioritizing antifibrotic therapy and incorporating a comprehensive treatment approach, patients with possible lung fibrosis can experience improved outcomes in terms of morbidity, mortality, and quality of life.
From the Research
Treatment Options for Possible Lung Fibrosis
- The treatment options for possible lung fibrosis include anti-fibrotic drugs such as nintedanib and pirfenidone, which have been shown to slow disease progression 2, 3, 4.
- Pirfenidone has been shown to stabilize and even improve lung function in some cases, although this is rare 3.
- Nintedanib and pirfenidone are the only effective therapeutics capable of slowing disease progression, and it is imperative that all treatment options are thoroughly evaluated and exhausted in each individual 2.
- Lung transplantation is also a treatment option for idiopathic pulmonary fibrosis (IPF), and it is the only treatment that has been shown to increase life expectancy 5.
- Other treatment approaches, such as anti-inflammatory drugs and immunosuppressants, have been used empirically, but their effectiveness has not been proven by large-scale randomized controlled trials 4, 6.
Management of Treatment
- Effectively managing anti-fibrotic treatment can be a challenge due to tolerability issues, the presence of pulmonary and extra-pulmonary comorbidities, and the need for concomitant medications in many patients 2.
- Practical recommendations for the management of nintedanib treatment in patients with IPF associated complications and those experiencing gastrointestinal side effects have been developed by a multidisciplinary panel of experts 2.
- The management of IPF requires a comprehensive approach, including the use of anti-fibrotic drugs, lung transplantation, and other treatment options, as well as the management of symptoms and comorbidities 5.