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The pathology and terminology of fibrosing alveolitis and the interstitial pneumonias

Department of Histopathology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK

Summary

• Cryptogenic fibrosing alveolitis (CFA) has traditionally related to a variety of histopathological patterns of interstitial pneumonia.
  
• Usual interstitial pneumonia (UIP) accounts for the majority of biopsied cases with the clinical label of CFA and is characterized by a temporally heterogenous histological pattern.
  
• Desquamative interstitial pneumonia (DIP) is characterized by an abundance of macrophages in alveoli and, unlike UIP, a temporally uniform pattern of interstitial inflammation and fibrosis. It is unlikely that DIP is an early (cellular) form of UIP.
  
• In non-specific interstitial pneumonia (NSIP), the histological pattern is again temporally homogenous, in distinction from UIP. Recent data suggest that a diagnosis of NSIP carries a better prognosis than patients with a pattern of UIP.
  

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