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Understanding Interstitial Lung Disease: A GP's Perspective

Join Dr. Nazia Chaudhuri, a respected respiratory physician, to demystify ILDs, their classifications, diagnostic criteria, and MDT diagnosis processes. Learn about the interstitium, clinical cases, and treatment implications. Don't miss the opportunity to enhance your ILD knowledge!

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Understanding Interstitial Lung Disease: A GP's 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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