Pharmaceutical Therapy
Current treatment options often involve the off-label use of anti-inflammatory drugs and are based on the concept that inflammation leads to injury and pulmonary fibrosis. Published guidelines suggest treatment with corticosteroids (prednisone or equivalent) and cytotoxic agents (azathioprine or cyclophosphamide) (1). The U.S. Food and Drug Administration has not yet approved any of these therapies for IPF because of a lack of clinical evidence of improved survival time or quality of life. This therapeutic approach to IPF has remained unchanged for 30 years, not because it is efficacious, but due to a lack of alternatives. One possible explanation for the lack of success of these drugs may be that fibrosis and T-helper (Th) cytokines—not inflammation—are the primary mechanisms responsible for the loss of lung function associated with IPF.
Lung Transplantation
Unless contraindications exist, lung transplantation should be considered for IPF patients with severe functional impairment, oxygen dependency, and a deteriorating condition. Early wait-listing is important, because time to procure a donor organ may exceed 2 years, and corrective treatment may be necessary to improve the patient's medical condition (11). Lung transplantation can reverse some of the symptoms and complications (e.g., pulmonary hypertension) associated with IPF; however, the 5-year survival rate after transplantation is only 50% to 60% (1).
Please see our Resources section for a list of lung transplantation Web sites.
Support Strategies
Because a healthy lifestyle may help slow disease progression, IPF patients should be encouraged to stop smoking, eat small and well-balanced meals, get adequate rest, and participate in an exercise regimen. They should enroll in a pulmonary physical rehabilitation program, if possible, and join a support group if one is available. Caregivers should also be encouraged to join a support group and/or to find other ways to help alleviate stress (e.g., exercise and relaxation techniques).
Experimental Treatments
Because existing IPF therapies are only marginally beneficial, alternative treatments are clearly needed.
Fortunately, recent advances in the understanding of the mechanisms underlying interstitial lung diseases (ILDs) are
leading to targeted interventions that may influence survival and quality of life. Researchers are evaluating a broad
range of potential interventions targeted toward various steps in the disease process. The following therapies are
currently under investigation in clinical trials:
- Interferon gamma (InterMune)
- N-acetylcysteine (N/A)
- Enbrel (Wyeth)
- Bosentan (Actelion)
- Gleevec (Novartis)
- Pirfenidone (InterMune)
- FG- 3019 (Fibrogen)
For an updated listing of clinical trials underway in the United States, as
well as other active IPF clinical research programs, please visit
http://www.coalitionforpf.org/IPFResearch/default.asp
One promising new treatment is interferon gamma-1b. This regulatory cytokine has antifibrotic and antifibrogenic effects and may regulate macrophage, fibroblast, and mast cell function; inhibit a variety of neutrophil-derived cytokines; and modify the balance of Th1 and Th2 cells in the lung (12,13). A multicenter Phase III trial is currently being conducted to confirm the effectiveness of interferon gamma-1b in slowing or reversing the scarring associated with IPF and potentially improving lung function and patient survival (Principal Investigator: Ganesh Raghu, MD; University of Washington Medical Center. Sponsor: InterMune, Inc.). For more information on this trial, click here.
Here is a partial list of other IPF trials/research programs currently under way:
- Genetic factors that may underlie pulmonary fibrosis (Principal Investigator: David Schwartz, MD; Duke University Medical Center). For more information on this trial, click here.
- Mechanisms of pulmonary fibrosis and factors that influence prognosis and predict survival in ILD patients (National Institutes of Health-funded Specialized Centers of Research at: National Jewish Medical and Research Center, Denver, CO [303-398-1621]; University of Michigan, Ann Arbor, MI [734-936-8917]; and Boston University Medical Center [617-638-4897]).
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