Physicians generally follow standards of care intended to improve symptoms and hopefully slow the progression of the disease. In 2001, a panel of experts sponsored by the American Thoracic Society and the European Respiratory Society recommended that if therapy was given, it should consist of a trial of corticosteroids and an immunosuppressive agent (e.g. azathioprine or cyclophosphamide). Recent evidence has suggested that adding a third therapy called N-acetylcysteine (NAC) to this regimen may be beneficial. It is important to recognize, however, that there are no definitive studies showing that this treatment approach is effective, and there is no consensus regarding the use of this approach in the pulmonary community. For a complete listing of active clinical trials for Pulmonary Fibrosis, please visit www.coalitionforpf.org.
Importantly, these therapies can cause side effects—some minor and some more serious. The potential risks and benefits of therapy should be discussed with your physician in detail. Be sure to contact your doctor or nurse if you have any negative reactions to any medications you have been prescribed.
In all cases, doctors should consider referring their patients to a clinical trial to gain access to experimental treatments and also refer patients for lung transplantation evaluation.
There remains no FDA-approved treatment for Pulmonary Fibrosis, and as of yet, there is no cure.
Please see the Lung Transplantation section of this educational tool
for more information, and consult your physician to determine if lung
transplantation is an option for you.
ARE THERE ANY NEW TREATMENTS ON THE HORIZON?
Researchers are developing and testing a variety of new ways to treat Pulmonary Fibrosis. These approaches target the various steps in the disease process. Therapies under investigation include:
- Antifibrotic or anti-fibrogenic agents to suppress the scarring process
- Antioxidants (such as N-acetylcysteine-NAC) to prevent damage to lung tissue
- Endothelin antagonists (such as certain medications for pulmonary arterial hypertension (PAH)
- Monoclonal antibodies to inhibit “bad” cytokines (protein growth factors, such as TGF-beta, TNF-Alpha, VEGF, or CTGF) that activate inflammation.
There are several emerging therapies currently in clinical trials for Pulmonary Fibrosis patients. Contact the CPF or visit www.coalitionforpf.org for a complete up-to-date listing of active investigational research. The National Institutes of Health (NIH) also offers information at www.clinicaltrials.gov for those interested in identifying Pulmonary Fibrosis-related research at medical centers across the United States. It represents the most up-to-date research information for patients including clinical trial sites, patient inclusion/exclusion criteria, and contact information for each trial.
As with any course of care, please consult your physician to determine what current treatment options, including participation in clinical trials, may be appropriate for you.
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