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Interstitial Lung Disease (From the GP Perspective) .

Interstitial Lung Disease (From the GP Perspective). Dr Nazia Chaudhuri. Consultant Respiratory Physician with specialist interest in ILD & Honorary Senior Lecturer. University Hospital of South Manchester/ University of Manchester. http://northwestipfmanchester.weebly.com.

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Interstitial Lung Disease (From the GP Perspective) .

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  1. Interstitial Lung Disease (From the GP Perspective). Dr Nazia Chaudhuri. Consultant Respiratory Physician with specialist interest in ILD & Honorary Senior Lecturer. University Hospital of South Manchester/ University of Manchester. http://northwestipfmanchester.weebly.com http://northwestipfmanchester.weebly.com

  2. http://northwestipfmanchester.weebly.com

  3. “What do you want to know about ILD?” • “I don’t need to know about ILD” • “It’s rare!” • “None of my patients have ILD” • “I need to know a lot of other things………………..” • “Asthma, COPD, Angina, Hypertension, Diabetes, Diverticulitis, Irritable bowel, Headache, Back pain…………the list is endless” • “I don’t need to know about ILD” http://northwestipfmanchester.weebly.com

  4. “These are all MYTHS, MYTHS, MYTHS!!!” PROVE IT!! Tell me when to think about it And when I think about it Tell me what to do about it…. And when you send me your letter Let me at least have an idea of what you’re talking about! http://northwestipfmanchester.weebly.com

  5. Prove it’s not that rare. • What are ILDs? ERS/ATS Classification – confusing nomenclature • Clinical case to illustrate the presentations, Investigations and management of the commonest ILD • Key Learning points from a GP perspective. http://northwestipfmanchester.weebly.com

  6. What is the interstitium? • Interstitium (blood vessels, lymphatics, connective tissue separating the alveoli) • Fibrosis and inflammation http://northwestipfmanchester.weebly.com

  7. It’s not that rare! • Demedts 2001 – US 15% OP visits and 100,000 admissions due to ILD. • Few population based studies – prevelance and incidence difficult to estimate http://northwestipfmanchester.weebly.com

  8. CFA • Cryptogenic Fibrosing Alveolitis. • There are over 100 ILDs http://northwestipfmanchester.weebly.com

  9. Classification of ILD’s/DPLD’s Diffuse Parenchymal Lung Diseases (DPLD’s) DPLD of known cause e.g. drugs, CTD’s Exposure EAA, asbestos Idiopathic Interstitial Pneumonias (IIP’s) Other forms of DPLD LAM, Eosinophilic Pneumonia, LCH Granulomatous e.g.sarcoidosis * Idiopathic Pulmonary Fibrosis (IPF/CFA) * Interstitial Pneumonia Other than IPF NSIP AIP DIP LIP RB-ILD COP http://northwestipfmanchester.weebly.com

  10. Idiopathic Pulmonary Fibrosis (IPF/CFA) * Interstitial Pneumonia Other than IPF NSIP AIP DIP LIP RB-ILD COP FIBROTICS ACUTE/SUBACUTE SMOKING RELATED IPF NSIP DIP RB-ILD AIP COP http://northwestipfmanchester.weebly.com

  11. Simple to complicated nomenclature • To better characterise the different fibrotic lung diseases • Presentation; Prognostic; treatment implications • Describes a pattern of disease on CT/Biopsy • Split into Known causes – Drugs; CTD; Birds; farmers • Unknown – Idiopathic • IPF commonest • To make respiratory doctors look clever http://northwestipfmanchester.weebly.com

  12. NHS Commissioning for ILD: http://northwestipfmanchester.weebly.com

  13. MDT Diagnosis Equality of Access Clinical Trials Pharmacological Treatment Patient Support Group Palliative Care Support local services Shared protocols/guidelines Raise Standards of Care http://northwestipfmanchester.weebly.com

  14. http://northwestipfmanchester.weebly.com

  15. Case X: • 65 year old male • Known COPD ETT 400yards normally • SOB progressive for 6 months • Dry cough • Smoker • PMH: MI 5 years ago • poor response to inhaler therapy • Little improvement with frusemide • No asbestos exposure • No eye problems; skin changes; dysphagia http://northwestipfmanchester.weebly.com

  16. Examination: • Completing sentences • Sats 92% • Nail clubbing • 25-50% • Bibasal Inspiratory Crepitations – Velcro like http://northwestipfmanchester.weebly.com

  17. CxR: http://northwestipfmanchester.weebly.com

  18. From a GP Perspective: • Over 45 years old • Persistent SOB • Persistent Cough • Velcro crackles • Normal or Restrictive Spirometry • THINK ILD • THINK IPF http://www.soundsofipf.co.uk/test-your-ear.html http://northwestipfmanchester.weebly.com

  19. What do we do: • Try NOT to diagnose IPF • HISTORY IS KEY • MDT Discussion: 50% local diagnosis is incorrect • Is it Connective tissue disease? • Could it be a hypersensitivity? • Drug reaction? http://northwestipfmanchester.weebly.com

  20. PULMONARY FUNCTION TESTS: http://northwestipfmanchester.weebly.com

  21. HRCT: http://northwestipfmanchester.weebly.com

  22. MDT Diagnosis? • Idiopathic Pulmonary Fibrosis http://northwestipfmanchester.weebly.com

  23. Diagnostic Criteria: • Exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity). • UIP pattern on HRCT in patients not subjected to surgical lung biopsy • Combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy • “The accuracy of the diagnosis of IPF increases with multidisciplinary discussion between pulmonologists, radiologists, and pathologists experienced in the diagnosis of ILD” • An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based guidelines for Diagnosis and Management. Rhagu et al. Am J Respir Crit Care Med Vol 183. pp 788–824, 2011. http://northwestipfmanchester.weebly.com

  24. Biospy: • NOT required if classical history and CT changes • Required if diagnosis uncertain, unusual CT appearance. http://northwestipfmanchester.weebly.com

  25. IPF • US approx 150,000IPF patients • 14-43 IPF cases per 100,000 • UK 8-9 per 100,000 • 5% increase incidence year on year http://northwestipfmanchester.weebly.com

  26. Median survival 2-3 years after diagnosis • Estimated 5 year survival 37% • Accurate and early diagnosis often delayed on average 1-2 years from onset of symptoms • Early symptoms non-specific and similar to other respiratory diseases http://northwestipfmanchester.weebly.com

  27. Disease course unpredictable and variable http://northwestipfmanchester.weebly.com

  28. Monitoring progression: • Worsening symptoms – subjective • Number of exacerbations • Shortens median survival • 50% die in hospital • Worsening fibrosis and honeycombing on CT http://northwestipfmanchester.weebly.com

  29. FVC decline >10% predictor of mortality 5x increase at one year • Recent evidence that 5-10% decline increased risk of mortality vs. stable. 1 year risk of death 2x higher • Am J RespirCrit Care Med. 2011 Dec 15;184(12):1382-9. doi: 10.1164/rccm.201105-0840OC. Epub 2011 Sep 22. • Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. • du Bois RM1, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, King TE Jr, Lancaster L, Noble PW, Sahn SA, Thomeer M, Valeyre D, Wells AU. • DLCO>15% http://northwestipfmanchester.weebly.com

  30. Treatment: http://northwestipfmanchester.weebly.com

  31. http://northwestipfmanchester.weebly.com

  32. Management of IPF: • Holistic approach • Education • Non-pharmacological • Pulmonary rehabilitation • Infection prevention • vaccinations • Palliative care • Oxygen • Comorbidities • Transplant in small minority • Pharmacological http://northwestipfmanchester.weebly.com

  33. http://northwestipfmanchester.weebly.com

  34. IPF 1.0 0.8 Probability of Survival 0.6 0.4 0.2 0 0 1 2 3 4 5 6 7 8 9 Years Azathioprine + prednisolone (n=14) Prednisolone (n=13) P = 0.02 (age adjusted) P = 0.16 Raghu G, et al. Am Rev Respir Dis. 1991;144:291-296. http://northwestipfmanchester.weebly.com

  35. http://northwestipfmanchester.weebly.com

  36. http://northwestipfmanchester.weebly.com

  37. Pirfenidone • Orally bioavailable synthetic molecule • Regulates the activity of TGFβ and TNFα in vitro • Inhibit fibroblast proliferation and collagen synthesis and reduce cellular and histological markers of fibrosis in animal models of lung fibrosis http://northwestipfmanchester.weebly.com

  38. Lots of RCTs: • Japanese Shinogi • CAPACITY 1 and 2 : European • ASCEND http://northwestipfmanchester.weebly.com

  39. Pirfenidone • Side effects • 98% reported a adverse event • 32% reported a serious adverse event • 17% discontinued treatment (vs 10% in placebo group) Biggest problem = cost (Euro 25,000-35,000) NICE says yes http://northwestipfmanchester.weebly.com

  40. 16.5% vs 31.8% http://northwestipfmanchester.weebly.com

  41. http://northwestipfmanchester.weebly.com 22.7 vs 9.7% no decline

  42. 48 and 68% reduction http://northwestipfmanchester.weebly.com

  43. NAC? http://northwestipfmanchester.weebly.com

  44. http://northwestipfmanchester.weebly.com

  45. http://northwestipfmanchester.weebly.com

  46. Nintedanib • Phase 2 TOMORROW http://northwestipfmanchester.weebly.com

  47. Phase 3 INPULSIS 1 and 2 • 52 week • 150mg bd • Multicentre • Reduce FVC decline • Reduce exacerbations • Diarrhoea 60% • Cost not known http://northwestipfmanchester.weebly.com

  48. http://northwestipfmanchester.weebly.com

  49. http://northwestipfmanchester.weebly.com

  50. ADULT LUNG TRANSPLANTATIONKaplan-Meier Survival By Diagnosis(Transplants: January 1990 – June 2006) ISHLT 2008 J Heart Lung Transplant 2008;27: 937-983

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