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Hamlet’s Delight New Guidelines for IPF

This resource provides evidence-based recommendations on diagnosis, management, and clinical follow-up for Idiopathic Pulmonary Fibrosis (IPF). It covers appropriate investigations, diagnostic algorithms, and treatment strategies for IPF patients to improve outcomes. The guidelines emphasize the importance of multi-disciplinary discussions for accurate diagnosis. By following these guidelines, healthcare professionals can enhance their approach to caring for individuals with IPF and potentially improve prognosis.

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Hamlet’s Delight New Guidelines for IPF

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  1. Hamlet’s DelightNew Guidelines for IPF CRC 2011 Ted Marras, MD FRCPC Toronto Western Hospital / University Health Network

  2. Declarations Potential conflicts of interest Financial • Study participation: Actelion, Boehringer-Ingelheim, Gilead, Intermune • Grant support: CPFF, CIHR Other • Clinical and academic interest in ILD Off label use of therapies None of the medications mentioned have a formal indication for treating IPF

  3. Considering revised guidelines for idiopathic pulmonary fibrosis (IPF): 1. Consider appropriate investigations and diagnostic algorithm for IPF 2. Select a management strategy that is most appropriate for a given IPF patient 3. Select an appropriate strategy of clinical follow-up for a given IPF patient Objectives

  4. What is it? Chronic, progressive fibrosis of the lung Unknown cause Why is it bad? Stiff lung  dyspnea Scarred lung  poor gas exchange Poor prognosis (difficult to quantify) IPF

  5. IPF - HRCT • Peripheral, basal predominant: • Reticulations, interlobular septal thickening, intralobular reticulations • Honeycombing

  6. IPF - Histology = UIP A) Heterogeneity, traction emphysema B) Subpleural fibrosis, fibroblast foci C) Fibroblast focus D) Microscopic honeycombing Raghu. Clin Chest Med 2004. 25(4)621-36.

  7. IPF - Natural history Raghu AJRCCM 183.788-824. 2011

  8. ATS / ERS / JRS / ALAT Provide evidence- based recommendations on diagnosis and management of IPF Joint Taskforce • 22 Pulmonary physicians • 4 Chest radiologists • 4 Lung pathologists • 3 Health care librarians • 1 Expert methodologist (respirologist) Raghu et al. AJRCCM 2011, 183:788-824

  9. Recommendations Reviewed published data Recommendations on questions • Direction – yes / no • Strength – strong / weak • Evidence quality Voted on by committee members

  10. Recommendations Raghu et al. AJRCCM 2011, 183:788-824

  11. Recommendations Raghu et al. AJRCCM 2011, 183:788-824

  12. Considering revised guidelines for idiopathic pulmonary fibrosis (IPF): 1. Consider appropriate investigations and diagnostic algorithm for IPF 2. Select a management strategy that is most appropriate for a given IPF patient 3. Select an appropriate strategy of clinical follow-up for a given IPF patient Objectives

  13. DiagnosisExcluding Connective Tissue Disease • Should a CTD serologic evaluation be performed in all people with suspected IPF? • No reliable data

  14. DiagnosisExcluding Connective Tissue Disease • Should a CTD serologic evaluation be performed in all people with suspected IPF? • No reliable data Even in absence of overt CTD: RF, anti-CCP, ANA (ENA – Jo-1, Scl-70, etc. may be helpful)

  15. DiagnosisUtility of BAL / TBBx • BAL may help differentiate HP • TBBx may help with granulomatous disorders • Should BAL / TBBx be performed in all people with suspected IPF?

  16. DiagnosisUtility of BAL / TBBx • BAL may help differentiate HP • TBBx may help with granulomatous disorders • Should BAL / TBBx be performed in all people with suspected IPF?

  17. DiagnosisMulti-disciplinary discussion (MDD) • IPF diagnosis usually requires expertise from clinicians, radiologists, pathologists • Proper communication increases inter-observer agreement • Should MDD be used in evaluating suspected IPF?

  18. DiagnosisMulti-disciplinary discussion (MDD) • IPF diagnosis usually requires expertise from clinicians, radiologists, pathologists • Proper communication increases inter-observer agreement • Should MDD be used in evaluating suspected IPF? • Not possible for many practitioners • Efforts to promote verbal communication should be made

  19. Diagnosis Consider: • Clinical • Radiology - HRCT • Histology - surgical lung biopsy

  20. DiagnosisHRCT Relevant features • Distribution subpleural / basal predominant • Reticulation • Honeycombing + traction bronchiectasis • Absence of inconsistent features: • Upper lobe predominant • Peribronchialpredominant • GGO > reticulation • Profuse micronodules • Discrete cysts – multiple, bilateral, away from HC • Diffuse mosaicism • Consolidation

  21. DiagnosisHRCT HRCT classification for suspected IPF • UIP pattern (1,2,3,4) • Possible UIP pattern (1,2,4) • Inconsistent with UIP (4 not fulfilled) • Subpleural / basal • Reticulation • Honeycombing + traction bronchiectasis • Absence of inconsistent features

  22. DiagnosisHistology Relevant features • Fibrosis + subpleural / paraseptal HC • Patchy • Fibroblast foci • Absence of inconsistent features: • Hyaline membranes • Organizing pneumonia • Granulomas • Marked inflammation away from HC • Predominantly airway centred

  23. DiagnosisHistology Histologic classification for suspected IPF • UIP pattern (1,2,3,4) • Probable UIP pattern (1 and [2 or 3] and 4) or HC only • Possible UIP pattern (1,4) • Not UIP pattern (4 not fulfilled) • Fibrosis + subpleural / paraseptal HC • Patchy • Fibroblast foci • Absence of inconsistent features

  24. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  25. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  26. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  27. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  28. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  29. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  30. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  31. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  32. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  33. DiagnosisHRCT /Histology * Multidisciplinary discussion recommended

  34. Diagnosis Suspected IPF yes Identifiable cause? Not IPF

  35. Diagnosis Suspected IPF yes Identifiable cause? Not IPF no HRCT UIP IPF

  36. Diagnosis Suspected IPF yes Identifiable cause? Not IPF no HRCT possible UIP or inconsistent with UIP UIP Surgical Biopsy IPF

  37. Diagnosis Suspected IPF yes Identifiable cause? Not IPF no HRCT possible UIP or inconsistent with UIP Not UIP UIP Surgical Biopsy IPF

  38. Diagnosis Suspected IPF yes Identifiable cause? Not IPF no HRCT possible UIP or inconsistent with UIP Not UIP UIP Surgical Biopsy UIP, probable, possible IPF See table

  39. Considering revised guidelines for idiopathic pulmonary fibrosis (IPF): 1. Consider appropriate investigations and diagnostic algorithm for IPF 2. Select a management strategy that is most appropriate for a given IPF patient 3. Select an appropriate strategy of clinical follow-up for a given IPF patient Objectives

  40. IPF Treatment

  41. IPF Treatment

  42. IPF Treatment * Small physiologic benefit, may have significant toxicities

  43. IPF Treatment * Limited data, safe, maybe cheap; preparation not standardized

  44. IPF Treatment * Supportive study, several limitations

  45. IPF Treatment

  46. IPF Treatment

  47. IPF Treatment

  48. IPF Treatment

  49. “Nonpharmacologic” Treatment

  50. Transplant Who to consider? • Discuss at diagnosis • Detailed evaluation: • Advanced at diagnosis • With objective deterioration

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