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Annotated IPF Guidelines: Management of IPF

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Annotated IPF Guidelines: Management of IPF

Current guidelines for IPF

An Official ATS/ERS/JRS/ALAT Statement: Evidence-Based Guidelines For Managing IPF. 

 

Excerpted from: Raghu G, Collard HR, Egan JJ et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

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Note: this document was published in 2011. A revised version was published in 2018. Certain aspects of this document may be out of date and caution should be used when applying these in clinical practice or other usages.

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.

 

Managing IPF

  • IPF is a fatal lung disease whose natural history is variable and unpredictable
  • Although nonpharmacologic strategies and comorbidity management have shown variable effect on patient outcomes, many of these approaches are recommended by the 2011 ATS/ERS/JRS/ALAT guidelines for patients with IPF

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    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Nonpharmacologic treatments for IPF

    Nonpharmacologic treatments for IPF
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    Oxygen Therapy1
    The guidelines recommend long-term oxygen supplementation for patients with IPF and clinically significant hypoxemia.
     
    Learn more in:Managing IPF: Symptoms
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Nonpharmacologic treatments for IPF (cont.)

    Nonpharmacologic treatments for IPF (cont.)
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    Lung Transplantation1
    The guidelines recommend lung transplantation for appropriate patients with IPF.
     
    Learn more in:Managing IPF: Symptoms
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Nonpharmacologic treatments for IPF (cont.)

    Nonpharmacologic treatments for IPF (cont.)
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    Mechanical Ventilation1
    The guidelines do not recommend mechanical ventilation for the majority of patients with IPF and respiratory failure.
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Nonpharmacologic treatments for IPF (cont.)

    Nonpharmacologic treatments for IPF (cont.)
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    Pulmonary Rehab1
    The guidelines recommend that the majority of patients with IPF receive pulmonary rehabilitation.
     
    Learn more in:Managing IPF: Symptoms
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Treating comorbidities

    Treating comorbidities
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    Treating Complications and Comorbidities1
    Treating comorbidities may or may not affect outcomes for patients with IPF. Check the guidelines for recommendations on treating individual comorbidities.
     
    Learn more in:Managing IPF: Comorbidities
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Palliative care

    Palliative care
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    Palliative Care1
    Individualized care should focus on relieving physical and emotional suffering, as well as psychological and spiritual support for patients and caregivers.
     
    It should be used as an adjunct to disease-based care.
    Learn more in:About IPF: Disease course
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Monitoring disease progression

    Monitoring disease progression
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    Disease Monitoring1
    Patients should be monitored regularly to detect worsening symptoms and oxygenation, as well as developing comorbidities (disease- or treatment-based).
     
    Learn more in:About IPF: Disease course
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Monitoring disease progression

    Monitoring disease progression
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    Disease Progression1
    Changes associated with disease progression include:
    • Progressive dyspnea
    • Progressive, sustained decrease from baseline in absolute FVC or Dlco
    • Progressive fibrosis from baseline on HRCT
    • Acute exacerbation
    • Death from respiratory failure
     
    Learn more in:About IPF: Disease course
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Monitoring for worsening symptoms

    Monitoring for worsening symptoms
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    Worsening Symptoms1
    Identifying patients with worsening symptoms is important to properly manage their treatment.
     
    Learn more in:About IPF: Disease course
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Monitoring for oxygenation

    Monitoring for oxygenation
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    Worsening Oxygenation1
    Monitoring oxygenation levels is critical to a timely prescription for supplemental oxygen.
    • Oxygenation should be measured at rest and exertion at baseline and follow-up appointments
    • Desaturation of ≤88% should generally lead to use of supplemental oxygen
     
    Learn more in:Managing IPF: Symptoms
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    Monitoring for comorbidities

    Monitoring for comorbidities
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    Monitoring Complications and Comorbidities1
    The guidelines do not provide recommendations for routine screening for comorbidities.
    • Acute respiratory worsening should lead to evaluation for AE-IPF
     
    Learn more in:Managing IPF: Comorbidities
    1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 

    References

    1.Raghu G et al. Am J Respir Crit Care Med2011; 183(6):788-824.
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