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Diagnosing idiopathic pulmonary fibrosis without a lung biopsy: honeycombing not required
  1. Robert Homer1,2,
  2. David J Lederer3
  1. 1Department of Pathology, Yale University, New Haven, Connecticut, USA
  2. 2Pathology and Laboratory Medicine Service, VA CT HeathCare System, West Haven, Connecticut, USA
  3. 3Departments of Medicine and Epidemiology, Columbia University Medical Center, New York, New York, USA
  1. Correspondence to Dr Robert Homer, Department of Pathology, Yale University, 310 Cedar St, New Haven, CT 06510, USA; robert.homer{at}

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Nearly 50 years ago, idiopathic interstitial pneumonias (IIPs) were defined by Liebow and Carrington based on the histological pattern present in the lung.1 ,2 Today, we consider histological patterns in the context of clinical and radiological information in order to diagnose a specific IIP, a paradigm shift initially introduced by a Consensus Statement published by the American Thoracic Society and European Respiratory Society in 2002 and developed further in 2011.3 With regard to idiopathic pulmonary fibrosis (IPF), this approach requires the presence of a usual interstitial pneumonia pattern (UIP), either on high resolution CT (HRCT) or histology or both, and the absence of any clinical history or morphological pattern that suggests another aetiology or another disease. The most recent iteration also introduced two new and related features. The first was a formal recognition that even in cases in which a biopsy was obtained, radiology had a role in modifying the interpretation of the pathological findings. In some cases, this meant that a definitive pathological diagnosis of IPF, once considered the gold standard, could be overridden by a negative radiological impression. In other cases, a diagnosis of IPF could be made even in the absence of definitive pathological evidence. The second major change was to introduce a multidisciplinary consensus conference as the mechanism for implementing the integration of pathology, radiology and clinical findings, such as in those cases just described in which there was substantial disagreement between pathology and radiology or those in which both evaluations were inconclusive. In many cases, however, pathology is still the definitive study especially in cases in which the …

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  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.

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