Idiopathic interstitial pneumonias

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  • 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
  • Rare minimal honeycombing
  • Subpleural sparing
  • Potentially reversible with immunosupression
  • Homogeneous
  • Can be cellular (GGO) and/or fibrotic (traction bronchiectasis"
  • Much better prognosis than UIP, esecially cellular NSIP

Cryptogenic organizing pneumonia (COP)

  • Patchy peripheral subpleural or peribronchovascular consolidation
  • Atoll sign, aka reverse halo
  • 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
  • aka BOOP
  • Usually present after treatment with antibiotics and persistent fever and opacities
  • Looks just like Eosinophillic pneumonia

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
  • Lower lobe predominant

Lymphoid Intersitial Pneumonia (LIP)

  • Groung glass opacities
  • Cystic lesions, generallt fewer in number (10-20)
  • Can be a/w sjogren.

Acute Interstial Pneumonia (AIP)

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


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