Difference between revisions of "Idiopathic interstitial pneumonias"

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Idopathic interstitial pneumonias (IIP) are rare, but common disorders such as [[sarcoidosis]], vasculitis and connective tissue disorders display an identicle morphologic pattern.  Bleomycin drug toxicity can also give a similar appearance.
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*Rare
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*Reaction to lung injury
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*Variable inflammation and fibrosis
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*Variable response to treatment
  
  
'''[[Idiopathic pulmonary fibrosis]] (IPF)''' - aka usual interstital pneumonia (UIP)
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Common disorders such as [[sarcoidosis]], vasculitis and connective tissue disorders display an identicle morphologic pattern.  Bleomycin drug toxicity can also give a similar appearance.
 +
 
 +
 
 +
'''[[Idiopathic pulmonary fibrosis]] (IPF)'''
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*Usual interstital pneumonia (UIP) is the histologic pattern
 
*Basal and peripheral reticular opacities
 
*Basal and peripheral reticular opacities
*'''Honeycombing'''
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*'''Honeycombing''', subpleural cysts
*Traction bronchiectasis
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*Traction bronchiectasis due to surrounding fibrosis
 
*Apicobasal gradient - gets worse from apex to base
 
*Apicobasal gradient - gets worse from apex to base
 
*Limited ground glass opacities
 
*Limited ground glass opacities
 
*Heterogeneous - diseased lung alternating with healthy lung
 
*Heterogeneous - diseased lung alternating with healthy lung
*Exclusion of known causes (toxins, environmental exposure, etc)
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*Exclusion of known causes (toxins, environmental exposure, collagen vascular disease, etc)
*No treatment available
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*Reduced lung volume on x-ray
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*Mean survival 3 years
  
  

Revision as of 17:49, 1 June 2012

  • Rare
  • Reaction to lung injury
  • Variable inflammation and fibrosis
  • Variable response to treatment


Common disorders such as sarcoidosis, vasculitis and connective tissue disorders display an identicle morphologic pattern. Bleomycin drug toxicity can also give a similar appearance.


Idiopathic pulmonary fibrosis (IPF)

  • Usual interstital pneumonia (UIP) is the histologic pattern
  • Basal and peripheral reticular opacities
  • Honeycombing, subpleural cysts
  • Traction bronchiectasis due to surrounding fibrosis
  • Apicobasal gradient - gets worse from apex to base
  • Limited ground glass opacities
  • Heterogeneous - diseased lung alternating with healthy lung
  • Exclusion of known causes (toxins, environmental exposure, collagen vascular disease, etc)
  • Reduced lung volume on x-ray
  • Mean survival 3 years


Nonspecific intersitial pneumonia (NSIP)

  • Basal ground glass opacitis predominate over reticulat opacities
  • UIP without honeycombing
  • Subpleural sparing
  • Potentially reversible with immunosupression


Cryptogenic organizing pneumonia (COP)

  • Patchy peripheral subpleural or peribronchovascular consolidation
  • Atoll sign
  • Idiopathic inflammation of the bronchioles and surrounding tissue causing granulation tissue and "non-infectious pneumonia"
  • Responds to steroids not antibiotics
  • a/w many systemic diseases (lupus, c-anca, GVHD, etc)
  • No pleural effusion or lymphadenopathy


Respiratory Bronchiolitis associated Interstitial Lung Disease (RB ILD)

  • Smoking related disease
  • Centrilobular nodules
  • Ground glass opacities
  • Upper lobe predominant


Desquamative Intersitial Pneumonia (DIP)

  • Smoking related disease
  • Centrilobular nodules
  • Ground glass opacities


Lymphoid Intersitial Pneumonia (LIP)

  • Groung glass opacities
  • Cystic lesions


Acute Interstial Pneumonia (AIP)

  • Diffuse lung consolidation
  • Groung glass opacities
  • Progress to fibrosis after acute phase


Links

Radiology Assistant webpage