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Interstitial Lung Disease |
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Idiopathic Pulmonary Fibrosis
 Signs and Symptoms
Diagnosis
Treatment
Treatment Unfortunately, there is currently no cure for idiopathic pulmonary fibrosis (IPF), and there is no proven medication therapy to treat it. Medical studies suggest a potential role for several therapies, listed below, but further study is needed.
- Pirfenidone — Data from three large studies of pirfenidone have suggested a benefit for IPF. Pirfenidone appears to slightly slow the decline in lung function. This drug is not currently approved for use in the United States but will be reviewed by the Federal Drug Administration (FDA) sometime in 2009.
- Acetylcysteine (NAC) — Data from a large study adding acetylcysteine to traditional medication therapies that include prednisone suggest it may help, but there's no consensus on whether or not the results are definitive. Acetylcysteine is available over the counter at most pharmacies and stores that carry supplements.
- Prednisone plus azathioprine — Combining prednisone with a second drug such as azathioprine, mycophenolate or cyclophosphamide has been used to treat IPF, but recent evidence suggests this approach isn't beneficial. If used to treat IPF, these medications should generally be combined with acetylcysteine, based on a recent study showing this combination to be more effective.
Ongoing studies of other medications for IPF have shown initial promise but need more research.
Pulmonary rehabilitation — a structured exercise and educational program designed for patients with chronic lung disease — is important for patients with IPF. We will also pay careful attention to any other medical problems associated with IPF, such as gastroesophageal reflux disease and pulmonary hypertension. Lastly, lung transplant may be an effective treatment option for some patients.
Reviewed by health care specialists at UCSF Medical Center.
Last updated January 13, 2010
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