• Characterised by pathological evidence of usual interstitial pneumonia (UIP)
  • M>F, onset around 50
  • Present with cough and dyspnoea
  • Fine end inspiratory crackles at the lung bases (like unzipping Velcro)
  • RHF
  • Around 3 year survival

 

2 – Non-specific interstitial pneumonia

  • M=F, presents around 40
  • Split into three types, 1 – associated predominantly with inflammation, 2 – associated with a mix of inflammation and fibrosis and 3 – predominantly fibrosis
  • Not associated with smoking

 

3 – Cryptogenic organising pneumonia

  • More common in non-smokers
  • Short preceding illness, generally follows what seems like and URT
  • Cough and dyspnoea
  • Associated with increased CRP and inflammatory markers
  • Give steroids – risk of relapse

 

4 – Acute

  • Histologically looks like ARDS
  • M=F, age 50ish, not associated with smoking
  • Associated with prior illness such as URTI
  • 50% mortality
  • Histologically associated with hyaline membranes

 

5 – Bronchiolitis associated interstitial pneumonia

  • Associated with smoking
  • Occurs more commonly in men age 40 – 50
  • Improves with smoking cessation
  • Not associated with clubbing

 

6 – Desquamative

  • Considered a form of bronchiolitis associated interstitial pneumonia that is more diffuse
  • Associated with intra-alveolar macrophage accumulation
  • Associated with clubbing
  • Good prognosis with smoking cessation and steroids

 

 

7 – Lymphoid interstitial

  • May be pre-neoplastic to lymphoma
  • F>M, age around 50
  • Associated with autoimmune diseases
  • Treat with steroids
 

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