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The impact of emphysema in pulmonary fibrosis

Vincent Cottin
European Respiratory Review 2013 22: 153-157; DOI: 10.1183/09059180.00000813
Vincent Cottin
*Hospices Civils de Lyon, Hôpital Louis Pradel, Service de Pneumologie – Centre de référence national des maladies pulmonaires rares, et centre de compétences de l’hypertension artérielle pulmonaire, Lyon, and #Université Claude Bernard Lyon 1, INRA, UMR754 INR-Vetagroup EPHR IFR 128, Lyon, France.
*Hospices Civils de Lyon, Hôpital Louis Pradel, Service de Pneumologie – Centre de référence national des maladies pulmonaires rares, et centre de compétences de l’hypertension artérielle pulmonaire, Lyon, and #Université Claude Bernard Lyon 1, INRA, UMR754 INR-Vetagroup EPHR IFR 128, Lyon, France.
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Abstract

Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.

  • Combined pulmonary fibrosis and emphysema
  • emphysema
  • idiopathic pulmonary fibrosis
  • pulmonary hypertension

Footnotes

  • Provenance

    Publication of this peer-reviewed article was supported by InterMune Inc, USA. (principal sponsor, European Respiratory Review issue 128).

  • Statement of Interest

    Conflict of interest information can be found alongside the online version of this article at err.ersjournals.com

  • Received March 4, 2013.
  • Accepted April 2, 2013.
  • ©ERS 2013
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The impact of emphysema in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Jun 2013, 22 (128) 153-157; DOI: 10.1183/09059180.00000813

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The impact of emphysema in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Jun 2013, 22 (128) 153-157; DOI: 10.1183/09059180.00000813
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