Epidemiology

• Mean Age of Onset: 40’s
• Relationship to Smoking: most cases are smokers

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Etiology

• Tobacco Abuse
• Connective Tissue Disease
• Eosinophilic Granuloma
• Asbestosis
• Gauchers Disease
• Niemann-Pick Disease
• Hermansky-Pudlak Syndrome
• Drugs
  • Amiodarone (see [[Amiodarone]])
  • Busulfan (see [[Busulfan]])
  • Interferon Alpha (see [[Interferons]])
  • Nitrofurantoin (see [[Nitrofurantoin]])
  • Rituximab (see [[Anti-CD20 Therapy]])
  • Sirolimus (see [[Sirolimus]])
  • Sulfasalazine (see [[Sulfasalazine]])
• Toxins
  • Aluminum (see [[Aluminum]])
  • Cobalt (see [[Cobalt]]): in combination with tungsten carbide, cobalt produces hard metal pneumoconiosis
• Idiopathic Desquamative Interstitial Pneumonia

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Clinical

• Insidious onset

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Treatment

• Good response to steroids (27% 12-year mortality rate)

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References

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