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The clinician's view of cryptogenic fibrosing alveolitis

Department of Respiratory Medicine, Green Lane Hospital, Green Lane, Auckland, New Zealand

Summary

• The advent and relatively widespread availability of CT has led to earlier diagnosis of CFA.
  
• The diagnostic accuracy of CT is considerably improved when a confident radiological diagnosis is made. Accumulated radiological expertise has led to a decreasing proportion of cases in whom a CT diagnosis is made with low confidence.
  
• CT is of value in the difficult clinical entity of "end-stage lung" where histological confirmation may not be realistic; a confident radiological diagnosis has an important clinical impact.
  
• CT appearances predict outcome as accurately as open lung biopsy in CFA. Treatment may be tailored according to the predominance of a reticular pattern, denoting fibrosis, or ground glass attenuation ("disease activity"), indicating a high likelihood of reversible disease.