Treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

IPF is a serious lung disease, the exact cause of which is not known. It generally affects people over 60 years of age and the main symptom is shortness of breath, which can have a considerable impact on day to day life. IPF was once thought to progress at a steady, predictable rate, but this is often not the case. Many people with IPF deteriorate rapidly, while others have periods of relative stability. In general people with IPF survive between two to five years. IPF is a difficult condition to manage. People with IPF often become very breathless and frequently have a dry cough. As a consequence this can also lead to difficulties undertaking activity and a heightened sense of anxiety. Treatments aim to reduce symptoms and improve survival. The type of treatment offered can vary, and people with IPF can also vary in their response to the available treatments. A number of new treatments for IPF have emerged over recent years. Of all the treatments now used in practice it is uncertain which are effective and provide the best value for money to the NHS. No existing systematic reviews investigate all of the treatments now available, nor do they also evaluate cost effectiveness. We aim to bring together the most up-to-date, high quality, published and unpublished evidence on the benefits, harms and costs of treatments for IPF. The results of our study will be helpful to patients and carers and also to health care professionals treating IPF. Our team is well placed for this research. We have vast experience of producing systematic reviews and of economic modelling. We also have clinical expertise in respiratory medicine, palliative care and physiotherapy. We will disseminate our findings through a final report and through submission of papers to relevant conferences and journals.