1. Pulmonary Fibrosis and Gradual Onset Breathlessness Dr. Tim Sutherland Consultant with a specialist interest in ILD

2. How do patients with pulmonary fibrosis present? When do I refer? What are the common causes of pulmonary fibrosis? How do we manage patients with pulmonary fibrosis? Overview – through the three concerns of my friend

3. Majority have progressive breathlessness over a period of time Other differentials include cardiac failure, COPD, obesity, deconditioning Associated medical conditions Drugs (www.pneumotox.com) Occupation Environment Smoking Family history Breathlessness and an approach to pulmonary fibrosis

4. 81 year old gentleman History of breathlessness and weight loss Past medical history of vascular disease Previous smoker Crackles on auscultation and was clubbed Spirometry shows a FVC of 86% Recent cases

5. How to interpret spirometry First concern

6. How to interpret spirometry Confirm the quality of the test As a general rule 80-120% predicated is normal Caution - treat generated reports with caution, especially if they say the unexpected First concern

7. Much more common than restriction Caused by a process that obstructs flow – generally in larger airways Typically causes reductions in FEV 1 and FEV 1 /FVC ratio Causes of obstructive defects: COPD Asthma Bronchiectasis/Cystic Fibrosis Obstruction

8. Restrictive defects are generally rare, esp in fibrosis Caused by a process that reduces all lung volumes - needs to be confirmed by a PFT lab Causes: (Intrinsic) - Interstitial lung diseases (Extrinsic) - Kyphoscoliosis - Ankylosing spondylitis - Muscular dystrophies Restriction

9. Interpretation can only be: - Normal - Obstructive - Restrictive (rare) - Undetermined Interpretation

11. 47 year old gentleman History of breathlessness over a year No past medical history Worked with aluminium, iron and steel Previous smoker Crackles on auscultation and was clubbed Spirometry shows a FVC of 98% Alternative presentation

13. What do I do with this? Second concern

14. If in doubt, refer Second concern

15. What causes pulmonary fibrosis?

16. Classification of Interstitial Lung Disease Interstitial Lung Disease OthersGranulomatous Disease Idiopathic Interstitial Pneumonias ILD of known cause Chronic fibrosing Smoking related Acute/ subacute Drugs CTD RA etc IPF NSIP RBILD DIP COP AIP LAM LCH etc

17. Classification of Interstitial Lung Disease Interstitial Lung Disease OthersGranulomatous Disease Idiopathic Interstitial Pneumonias ILD of known cause Chronic fibrosing Smoking related Acute/ subacute Drugs CTD RA etc IPF NSIP RBILD DIP COP AIP LAM LCH etc

18. One of the most frequent diagnoses – approximately 30 cases per 100,000 Used to be called CFA No known cause Tends to be older people, men more than women No cure but there is treatment Idiopathic Pulmonary Fibrosis

19. Lung cancer survival IPF survival Thank god it’s not lung cancer Doctor!

20. Lung cancer survival IPF survival Thank god it’s not lung cancer Doctor!

21. Lung cancer survival IPF survival Thank god it’s not lung cancer Doctor!

22. Lung cancer survival IPF survival Main management is: - symptom control - oxygen assessment - pulmonary rehabilitation - planning for the future/managing expectations - transplant? - newer medication Thank god it’s not lung cancer Doctor!

23. Antifibrotic agent – currently a red drug Up to 9 capsules a day Significant side effects – liver, sun-burn, GI Requires LFT monitoring We currently have about 110 patients on this drug Pirfenidone

24. Tyrosine kinase inhibitor – currently named patient only 2 capsules a day Fewer side effects Requires LFT monitoring Nintedanib

25. If in doubt, call us or the specialist nurses Control symptoms, check oxygen and discuss patient expectations Initiate future planning when able to Third concern – how do I co-manage these patients?

26. www.blf.org.uk www.actionpulmonaryfibrosis.org Any questions?