Excerpt

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease (ILD) of unknown origin. It is a difficult disease to diagnose and often requires the collaborative expertise of a chest physician, radiologist and histopathologist to reach a consensus diagnosis. Most people with idiopathic pulmonary fibrosis experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death. Specific pharmacological therapies for IPF are limited but the last decade has seen more trials of new drugs which have had a variable impact on clinical practice. A number of difficulties arise when undertaking clinical trials in IPF in terms of defining precise, diagnostic inclusion criteria and clinically meaningful end-points. However, such trials are the only way by which promising new treatments will come to benefit patients. Furthermore, it is only by performing rigorous clinical trials, we have learned that drugs once widely used to treat IPF may in fact have been harmful. The limitations of current pharmacological therapies for IPF highlight the importance of other forms of treatment including lung transplantation and best supportive care such as oxygen therapy, pulmonary rehabilitation and palliation of symptoms. These are interventions which justifiably require scrutiny in the context of healthcare delivery by the modern NHS. Despite the significant burden of disease caused by IPF, there is currently no established framework within the NHS for its diagnosis and management thus creating an environment in which significant variations in clinical care may occur. In recognition of this, the Department of Health commissioned the National Institute of Health and Care Excellence (NICE) to produce a guideline aimed at improving the care of people with IPF.

Contents

• Acknowledgments
• 1. Guideline development group members
• 2. Introduction
  • Why the guideline is needed
  • Terminology and definitions
  • Epidemiology of IPF
  • The natural history of IPF
  • What is in this guideline?
  • What is not in this guideline?
• 3. Development of the guideline
  • 3.1. What is a NICE clinical guideline?
  • 3.2. Remit
  • 3.3. Who developed this guideline?
  • 3.4. What this guideline covers
  • 3.5. What this guideline does not cover
  • 3.6. Relationships between the guideline and other NICE guidance
• 4. Methodology
  • 4.1. Developing the review questions and outcomes
  • 4.2. Searching for evidence
  • 4.3. Evidence of effectiveness
  • 4.4. Evidence of cost-effectiveness
  • 4.5. Developing recommendations
• 5. Guideline summary
  • 5.1. Algorithms
  • 5.2. Key priorities for implementation
  • 5.3. Full list of recommendations
  • 5.4. Key research recommendations
• 6. Diagnosis
  • 6.1. Review introduction
  • 6.2. Clinical questions and review methodology
  • 6.3. Clinical evidence
  • 6.4. Economic evidence summary
  • 6.5. Recommendations and link to evidence
• 7. Prognosis
  • 7.1. Review introduction
  • 7.2. Clinical questions and review methodology
  • 7.3. Clinical evidence
  • 7.4. Study Quality
  • 7.5. Do serial pulmonary function tests (PFTs) (resting spirometric, gas transfer measurement and oxygen saturation) predict prognosis of IPF?
  • 7.6. Does baseline sub-maximal exercise testing predict prognosis of IPF?
  • 7.7. Does baseline echocardiography predict prognosis of IPF?
  • 7.8. Do baseline CT scores predict prognosis of IPF?
  • 7.9. Economic evidence
  • 7.10. Evidence statements
  • 7.11. Recommendations and link to evidence
• 8. Pulmonary rehabilitation
  • 8.1. Review introduction
  • 8.2. Clinical questions and review methodology
  • 8.3. Clinical evidence
  • 8.4. Economic evidence
  • 8.5. Evidence statements
  • 8.6. Recommendations and link to evidence
• 9. Best supportive care
  • 9.1. Review Introduction
  • 9.2. Clinical question and review methodology
  • 9.3. Clinical evidence
  • 9.4. Economic evidence
  • 9.5. Evidence statements
  • 9.6. Recommendations and link to evidence
• 10. Psychosocial support
  • 10.1. Review Introduction
  • 10.2. Clinical question and review methodology
  • 10.3. Clinical evidence
  • 10.4. Economic evidence
  • 10.5. Evidence statements
  • 10.6. Recommendations and link to evidence
• 11. Pharmacological interventions
  • 11.1. Review Introduction
  • 11.2. Clinical questions and review methodology
  • 11.3. Clinical evidence
  • 11.4. Economic evidence summary
  • 11.5. Evidence statements
  • 11.6. Recommendations and link to evidence
• 12. Lung transplantation
  • 12.1. Review introduction
  • 12.2. Clinical question and review methodology
  • 12.3. Clinical evidence
  • 12.4. Economic evidence
  • 12.5. Evidence statements
  • 12.6. Recommendations and link to evidence
• 13. Ventilation
  • 13.1. Review introduction
  • 13.2. Clinical question and review methodology
  • 13.3. Clinical evidence
  • 13.4. Economic evidence
  • 13.5. Evidence statements
  • 13.6. Recommendations and link to evidence
• 14. Review and follow-up
  • 14.1. Review introduction
  • 14.2. Clinical question and review methodology
  • 14.3. Clinical evidence
  • 14.4. Economic evidence
  • 14.5. Evidence statements
  • 14.6. Recommendations and link to evidence
• 15. Reference list
• 16. Acronyms and abbreviations
• 17. Glossary
• Appendices
  • Appendix A. Scope
  • Appendix B. Declarations of interest
  • Appendix C. Review protocols
  • Appendix D. Literature search strategy
  • Appendix E. Forest plots
  • Appendix F. Clinical evidence tables
  • Appendix G. Economic evidence tables
  • Appendix H. Interpreting post-test probabilities by considering prevalence/pre-test probability
  • Appendix I. Calculations of standard errors from HR, RR and ORs
  • Appendix J. Costing of a Multidisciplinary Team (MDT) in the Context of an Interstitial Lung Disease (ILD) Network: Finding the incremental cost of involving an MDT in the IPF diagnostic pathway
  • Appendix K. Placing the diagnostic clinical evidence into an economic framework for decision making
  • Appendix L. Cost-effectiveness analysis – Pulmonary rehabilitation for patients with Idiopathic Pulmonary Fibrosis
  • Appendix M. Model produced Median and Mean Life Expectancies for people diagnosed with IPF
  • Appendix N. Adverse events table
  • Appendix O. The cost of pharmacological interventions for IPF
  • Appendix P. Research recommendations
  • Appendix Q. Adapted Prisma Diagrams
  • Appendix R. Excluded Studies
  • Appendix S. Reference list

Disclaimer: Healthcare professionals are expected to take NICE clinical guidelines fully into account when exercising their clinical judgement. However, the guidance does not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of each patient, in consultation with the patient and/or their guardian or carer.