The spectrum of diseases collectively known as pulmonary fibrosis does not have reliable therapeutic options. Many treatment protocols have been the subject of clinical trials, resulting in few significant improvements in outcome. Recently, cellular mechanisms have been the focus of investigations in progenitor cell studies, suggesting an immunomodulatory role for mesenchymal stem cells. The possible role of fibrogenic cytokines and the use of antagonist molecules are promising for future therapies. A range of anti-inflammatory treatments, including macrolide antibiotics, may also hold promise. When medical therapy has been unsuccessful in altering the course of the disease, lung transplantation may offer a significant survival advantage. Improvement in pre-transplant assessment and postoperative care, combined with more effective immunosuppressive regimes, has seen survival rates of 40% after 5 years. New options for therapy will lead to improved survival in coming years.
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Competing interests: None.
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