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Annotated IPF Guidelines

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Annotated IPF Guidelines

Current guidelines for diagnosing IPF

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Diagnosis of Idiopathic Pulmonary Fibrosis: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.

 

Raghu G, Remy-Jardin M, Myers JL et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

Note: text in italics are excerpts from Raghu G, Remy-Jardin M, Myers JL et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

 

Definition of IPF

Definition of IPF
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IPF (n.)2,3: a chronic, progressive form of fibrotic interstitial pneumonia of unknown cause; associated with UIP patterns on HRCT and histopathology; requires exclusion of other forms of IP.
 
Learn more in:About IPF: Disease Course
HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IP; interstitial pneumonia; UIP, usual interstitial pneumonia.
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-664. 3. ATS/ERS. Am J Respir Crit Care Med. 2002;165(2 Pt 1):277-304.

Clinical presentation

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Clinical Presentation1,4,5
  • Chronic, exertional dyspnea
  • Cough
  • Bibasilar crackles
  • Digital clubbing
 
Learn more in:Diagnosing IPF: Clinical Presentation
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 4. King TE, Jr. et al. Am J Respir Crit Care Med. 2001;164(7):1171-1181. 5. Douglas WW et al. Am J Respir Crit Care Med. 2000;161(4 Pt 1):1172-1178.
Clinical Presentation
[IPF] should be considered in all adult
patients with unexplained chronic exertional dyspnea, cough, bibasilar inspiratory crackles, and/or digital clubbing.
 
The incidence of IPF increases with older age, with presentation typically occurring in the sixth and seventh decades.
 
Patients with IPF aged less than 50 years are rare; such patients may subsequently manifest overt features of an underlying connective tissue disease that was subclinical at the time IPF was diagnosed.
 
More men have been reported with IPF than women, and the majority of patients have a history of cigarette smoking.

Diagnostic criteria for IPF

Diagnostic criteria for IPF
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Diagnostic Criteria1
1.Exclude other potential causes of ILD
2.UIP pattern on HRCT
3.Specific combinations of HRCT and histopathologic patterns
 
Learn more in:Diagnosing IPF: Diagnostic criteria and algorithm
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

Medical history

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Question: Should patients with newly detected ILD of unknown cause who are clinically suspected of having IPF undergo a detailed, prompted history to exclude potential causes of the ILD?

 

The guideline panel recognized

there is no reasonable alternative to the

proposed course of action, so a motherhood

statement was made to take a detailed

history of medication use and

environmental exposures at home, work,

and other places that the patient frequently

visits, to identify or exclude potential causes

of ILD (e.g., hypersensitivity pneumonitis,

pneumoconiosis, drug toxicity).

Medical history

Patients with suspected IPF should have a detailed history of medications, exposures, and family history performed to rule out other causes of ILD.1

 

Learn more in:Diagnosing IPF: Clinical Features

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

Serologic testing

Serologic testing
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Serologic Testing
Ruling out other known causes of ILD (eg, CTD) is critical to diagnosing IPF1
 
The guidelines recommend performing testing for CTD only in patients with positive clinical manifestations, serologies, or other characteristics atypical for IPF.1
 
Learn more in:Diagnosing IPF: Clinical Signs
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.
CTD, connective tissue disease.

Bronchoalveolar lavage

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Question: Should patients

with newly detected ILD of unknown

cause who are clinically suspected

of having IPF undergo cellular

analysis of their BAL fluid?

 

Recommendation: For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and

have an HRCT pattern of probable UIP, indeterminate, or an alternative diagnosis, we suggest cellular analysis of their BAL fluid (conditional recommendation, very low quality of evidence).

Bronchoalveolar lavage1

Patients with suspected ILD who have a pattern other than UIP on HRCT may benefit from analysis of BAL fluid to rule out potential other ILDs, most notably eosinophilic pneumonia and sarcoidosis.

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

BAL, bronchoalveolar lavage.

HRCT patterns in IPF

HRCT patterns in IPF
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HRCT diagnostic categories1,6
  • UIP
  • Probable UIP
  • Indeterminate for UIP
  • Alternative diagnosis
 
Learn more in:Diagnosing IPF: Radiology
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 6.Lynch DA et al. Lancet. 2018;6(2):138-153.

HRCT: UIP pattern

HRCT: UIP pattern
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PPV, positive predictive value.
UIP Pattern1,6
  • Honeycombing with or without traction bronchiectasis or bronchiolectasis
  • Basal, peripheral distribution
  • Often heterogeneous distribution
A definite UIP pattern on HRCT has a 90%-100% PPV for IPF7-12
 
Learn more in:Diagnosing IPF: Radiology
1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 6. Lynch DA et al. Lancet. 2018;6(2):138-153. 7. Mathieson JR et al. Radiol. 1989;171(1):111-116. 8. Hunninghake GW et al. Am J Respir Crit Care Med. 2001;164(2):193-196. 9. Raghu G et al. Chest. 1999;116(5):1168-1174. 10. Grenier P et al. Radiology. 1991;179(1):123-132. 11. Lee KS et al. Radiology. 1994;191(3):669-673. 12. Swensen SJ et al. Radiology. 1997;205(1):229-234.

Surgical lung biopsy

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For HRCT patterns other than UIP:1

  • SLB is suggested because specific combinations of HRCT and histopathologic patterns are required for diagnosis of IPF when UIP is not present on HRCT

    •  

    1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

    Question: For patients with newly detected ILD of unknown cause who are clinically suspected of having IPF, should SLB be performed to ascertain the histopathology diagnosis of UIP pattern?

     

    Recommendation: For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable UIP, indeterminate, or an alternative diagnosis, we suggest SLB.

    Histopathologic patterns in IPF

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    Histopathologic patterns for diagnosing IPF

    The guidelines recommend the use of 4 categories for histopathologic classification in diagnosing IPF.

     

    The presence of UIP pattern on histopathology when combined with the exclusion of other known causes of ILD is likely to indicate IPF.  

     

    Other histopathologic patterns should be considered with the corresponding HRCT patterns and clinical information to help inform the most likely differential diagnosis.

     

     

     

    Histopathologic diagnostic categories1,6

  • UIP
  • Probable UIP
  • Indeterminate for UIP
  • Alternative diagnosis

    •  

    Learn more in:Diagnosing IPF: Radiology

    1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 6.Lynch DA et al. Lancet. 2018;6(2):138-153.

    Histopathology: UIP pattern

    Histopathology: UIP pattern
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    UIP pattern1,6
    • Dense fibrotic pattern with destructive scarring, with or without honeycombing
    • Predominant distribution of fibrosis: subpleural  and/or paraseptal
    • Patchy lung parenchyma involvement due to fibrosis
    • Fibroblast foci
    • No features present to suggest alternate diagnosis
    Learn more in:Diagnosing IPF: Histopathology
    1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 6. Lynch DA et al. Lancet. 2018;6(2):138-153.

    Suspected Case of IPF

     

    Histopathology

     

     

     

     

     

    UIP

    Probable UIP

    Indeterminate for UIP

    Alternative Diagnosis

    HRCT results

    UIP

    IPF

    IPF

    IPF

    Not IPF

     

    Probable UIP

    IPF

    IPF

    IPF Likely

    Not IPF

     

    Indeterminate for UIP

    IPF

    IPF Likely

    Indeterminate

    Not IPF

     

    Alternative Diagnosis

    IPF (likely) or non-IPF

    Not IPF

    Not IPF

    Not IPF

    Combinations of radiologic and pathologic data can point to a diagnosis1

    1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

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  • A UIP pattern on HRCT in combination with a thorough clinical history is diagnostic for IPF
  • In cases where HRCT does not show a UIP pattern, certain combinations of HRCT patterns and histopathologic patterns can help determine the likelihood that a patient has IPF

    • Adapted with permission from Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

    Multidisciplinary discussion

    Multidisciplinary discussion
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    MDD1
    The guidelines recommend the use of an MDD to guide decision-making in cases of suspected IPF.
     
    Learn more in:Diagnosing IPF: MDD
    MDD, multidisciplinary discussion.
    1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

    References

    1.Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.
    2.ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-664.
    3.ATS/ERS. Am J Respir Crit Care Med. 2002;165(2 Pt 1):277-304.
    4.King TE, Jr. et al. Am J Respir Crit Care Med. 2001;164(7):1171-1181.
    5.Douglas WW et al.Am J Respir Crit Care Med. 2000;161(4 Pt 1):1172-1178
    6.Lynch DA et al. Lancet. 2018;6(2):138-153.
    7.Mathieson JR et al. Radiol. 1989;171(1):111-116.
    8.Hunninghake GW et al. Am J Respir Crit Care Med. 2001;164(2):193-196.
    9.Raghu G et al. Chest. 1999;116(5):1168-1174.
    10.Grenier P et al. Radiology. 1991;179(1):123-132.
    11.Lee KS et al. Radiology. 1994;191(3):669-673.
    12.Swensen SJ et al. Radiology. 1997;205(1):229-234.

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