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Hamlet’s Delight New Guidelines for IPF

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Presentation on theme: "Hamlet’s Delight New Guidelines for IPF"— Presentation transcript:

1 Hamlet’s Delight New 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 Objectives 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 What’s presented here will undoubtedly have omissions, possibly small deviations from document to come

4 IPF 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) What’s presented here will undoubtedly have omissions, possibly small deviations from document to come

5 IPF - HRCT Peripheral, basal predominant:
Reticulations, interlobular septal thickening, intralobular reticulations Honeycombing Woman, 70s, longstanding dry cough and dyspnea

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

7 IPF - Natural history Raghu AJRCCM

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:

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:

11 Recommendations Raghu et al. AJRCCM 2011, 183:

12 Objectives 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 What’s presented here will undoubtedly have omissions, possibly small deviations from document to come

13 Diagnosis Excluding Connective Tissue Disease
Should a CTD serologic evaluation be performed in all people with suspected IPF? No reliable data UIP may occure in any CTD and may present before overt CTD

14 Diagnosis Excluding Connective Tissue Disease
Should a CTD serologic evaluation be performed in all people with suspected IPF? No reliable data Question Recommendation Vote Yes/No/Abs Direction Strength Evidence quality CTD serology? Yes Weak Very low 23/0/0 Even in absence of overt CTD: RF, anti-CCP, ANA (ENA – Jo-1, Scl-70, etc. may be helpful)

15 Diagnosis Utility 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 Diagnosis Utility 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? Question Recommendation Vote Yes/No/Abs Direction Strength Evidence quality BAL? No Weak Low 4/18/1 TBBx? 0/23/0

17 Diagnosis Multi-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? Operationalizing this outside of highly specialized centres is a challenge

18 Diagnosis Multi-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? Question Recommendation Vote Yes/No/Abs Direction Strength Evidence quality MDD? Yes Strong Low 0/23/0 Operationalizing this outside of highly specialized centres is a challenge Not possible for many practitioners Efforts to promote verbal communication should be made

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

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

21 Diagnosis HRCT 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 Diagnosis Histology 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 Diagnosis Histology 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 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Consistent with UIP (lack HC / traction bronchiectasis) UIP / Probable Possible UIP Probable* Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

25 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) Not UIP Yes No Consistent with UIP (lack HC / traction bronchiectasis) UIP / Probable Possible UIP Probable* Inconsistent with UIP (inconsistent features) All others Possible* Subpleural / basal, Reticulation, Honeycombing + traction bronchiectasis, Absence of inconsistent features * Multidisciplinary discussion recommended

26 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes Consistent with UIP (lack HC / traction bronchiectasis) UIP / Probable Possible UIP Probable* No Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

27 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Consistent with UIP (lack HC / traction bronchiectasis) UIP / Probable Possible UIP Probable* Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

28 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* May lack patchiness or HC * Multidisciplinary discussion recommended

29 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* Fibrosis only * Multidisciplinary discussion recommended

30 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

31 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

32 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* * Multidisciplinary discussion recommended

33 Diagnosis HRCT / Histology
Surgical biopsy IPF? UIP Not done (clinically typical) UIP / Probable / Possible Not UIP Yes No Possible UIP (lack HC) UIP / Probable Probable* Inconsistent with UIP (inconsistent features) All others Possible* Large proportion will have “possible” HRCT (no HC) and “possible” SLBx (fibrosis w/o alternate cause); Lots of MDDs! * Multidisciplinary discussion recommended

34 Diagnosis Suspected IPF yes Identifiable cause? Not IPF

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

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

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

38 Diagnosis Suspected IPF Identifiable cause? Not IPF HRCT
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 Objectives 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 Early treatment refers to medical therapy meant to modify IPF disease course

40 IPF Treatment Treatment Recommendation Vote Yes/No/Abs Direction
Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 Maybe should look carefully at the ones for which there is a weak recommendation against

41 IPF Treatment Treatment Recommendation Vote Yes/No/Abs Direction
Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 Maybe should look carefully at the ones for which there is a weak recommendation against

42 IPF Treatment Steroid + Aza + NAC* Weak 3 / 17 / 3
Recommendation Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC* Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 IFIGENIA - No diff in mortality or dyspnea, HRQL, radiology; 30% drop-out; unclear significance of the observed effect; no placebo * Small physiologic benefit, may have significant toxicities

43 IPF Treatment NAC alone* 5 / 15 / 3
Recommendation Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone* 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 IFIGENIA, some supportive uncontrolled data, safe; BUT preparation not standardized * Limited data, safe, maybe cheap; preparation not standardized

44 IPF Treatment Anticoagulation* 1 / 20 / 2
Recommendation Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation* 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 Supportive study; not blinded, differential drop-outs; failure to exclude PE * Supportive study, several limitations

45 IPF Treatment Pirfenidone Low-mod 4 / 10 / 17 Treatment Recommendation
Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17

46 IPF Treatment Pirfenidone Low-mod 4 / 10 / 17 Treatment Recommendation
Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 But incomplete data set

47 IPF Treatment Pirfenidone Low-mod 4 / 10 / 17 Treatment Recommendation
Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 But highly selective enrollment (desat on unvalidated ex test), primary endpoint changed part-way through

48 IPF Treatment Pirfenidone Low-mod 4 / 10 / 17 Treatment Recommendation
Vote Yes/No/Abs Direction Strength Evidence quality Steroids alone No Strong Very low 0 / 21 / 2 Colchicine Cyclosporine 0 / 18 / 4 Steroid + Aza / CY Low Steroid + Aza + NAC Weak 3 / 17 / 3 NAC alone 5 / 15 / 3 IFN gamma High 0 / 17 / 6 Bosentan Moderate 0 / 10 / 13 Etanercept Anticoagulation 1 / 20 / 2 Pirfenidone Low-mod 4 / 10 / 17 Voting by subsequent electronic polling to included the 2 latter studies: to be reviewed in Dr. Fell’s presentation

49 “Nonpharmacologic” Treatment
Recommendation Vote Yes/No/Abs Direction Strength Evidence quality Pulmonary rehabilitation Yes Weak Low 19 / 0 / 3 Oxygen Strong Very low 18 / 0 / 4 Transplantation 21 / 0 / 1 Rehab – long-term benefit unclear; O2 – for resting hypoxemia, leaving exertional up for debate; Tx – timing critical; discuss at Dx and detailed eval at 1st sign of objective deterioration

50 Transplant Who to consider?
Discuss at diagnosis Detailed evaluation: Advanced at diagnosis With objective deterioration Fairly liberal criteria to consider for assessment / transplant

51 Transplant Who to consider?
Discuss at diagnosis Detailed evaluation: Advanced at diagnosis With objective deterioration Baseline Severe dyspnea DLCO<40% 6MW SaO2 < 88% Extensive HC on HRCT Longitudinal Increasing dyspnea FVC decrease >10%* DLCO decrease >15%* Progression on HRCT Fairly liberal criteria to consider for assessment / transplant * Absolute measure

52 Additional Treatment - Acute exacerbations AEIPF - Definition
“Acute, clinically significant deterioration of unidentifiable cause in a patient with underlying IPF” Diagnostic Criteria IPF with unexplained worsening < 30 days HRCT new bilateral GGO and/or consolidation superimposed on typical IPF pattern No infection by tracheal aspirate or BAL Exclude: CHF, PE, identifiable acute lung injury cause Median mortality among series

53 Additional Treatment Treatment Recommendation Vote Yes/No/Abs
Direction Strength Evidence quality Steroids in AEIPF Yes Weak Very Low 14 / 5 / 1 Mechanical ventilation No Low 2 / 19 / 1 Pulmonary hypertension Very low 8 / 14 / 1 Asymptomatic GERD 15 / 8 / 0

54 Objectives 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 What’s presented here will undoubtedly have omissions, possibly small deviations from document to come

55 Monitoring for progression
Routine PFT Sustained change in absolute: FVC of 10% (e.g. 2L1.8L)* DLCO of 15% (Both associated with mortality, suggestive of progression) *Smaller progressive, sustained changes MAY be relevant (e.g. 5-10% FVC decline)

56 Sunset George Lake, Killarney Provincial Park, Aug 2005

57 Monitoring for progression
Routine PFT Sustained change in absolute: FVC of 10% (e.g. 2L1.8L) DLCO of 15% (Both associated with mortality, suggestive of progression) 6MW distance / oximetry too variable over long time periods (good discriminative test, not a good evaluative test) Discriminative test – accurately distinguishes the sick from the well; or discriminates by illness severity Evaluative test – accurately evaluates changes over time (natural history or interventions)

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