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Thorax. 2007 Nov;62(11):1008-12.

Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate.

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  • 1Interstitial Lung Disease Unit, Department of occupational and Environmental Medicine, National Heart and Lung Institute, Imperial College of Science, Technology and Medicine, London, UK.


Among the idiopathic interstitial pneumonias, the two entities-idiopathic pulmonary fibrosis (IPF) characterised by the presence of the usual interstitial pneumonia pattern of histopathology (IPF/UIP) and non-specific interstitial pneumonia (NSIP; same nomenclature for the histopathological pattern and idiopathic disease) - have provoked considerable debate. IPF/UIP and NSIP closely mimic each other clinically but NSIP has a far better outcome. However, it remains unclear if NSIP is a truly separate and distinct entity. The histopathological pattern of NSIP can be found in a wide variety of clinical and radiological contexts. This review addresses these and other uncertainties regarding NSIP and UIP.

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