IPF, comorbidities and management implications

IPF, comorbidities and management implications

Authors

  • Carlo Vancheri Regional Centre for Rare Pulmonary Diseases, Department of Clinical and Experimental Medicine, University of Catania, Italy
  • Vincent Cottin National Reference Center for Rare Pulmonary Diseases, University of Lyon, France
  • Michael Kreuter Centre for Rare and Interstitial Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, and Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL), Germany
  • Ole Hilberg Ole Hilberg, Århus Universitetshospital, Århus, Denmark

Keywords:

idiopathic pulmonary fibrosis, comorbidity, cardiovascular disease, lung cancer, pulmonary hypertension

Abstract

Idiopathic pulmonary fibrosis (IPF) is a complex disease that is associated with various respiratory and non-respiratory comorbidities. The most common comorbidity is cardiovascular disease (CVD), which increases in incidence with increasing duration of IPF and is associated with a higher risk of mortality. The direction of causality between CVD and IPF is unclear. There is evidence that IPF is associated with a prothrombotic state; however, warfarin is not beneficial in IPF patients. Lung cancer is another common comorbidity, being present in more than 50% of IPF patients at 10 years after diagnosis. IPF and lung cancer share several risk factors and pathogenic pathways and also show a similar anatomic distribution; this can make radiological diagnosis difficult. As with CVD, lung cancer in IPF patients is associated with a significantly worse prognosis and treatment options are limited. Surgery, chemotherapy, and radiotherapy have all been associated with an increased risk of morbidity and mortality. However, treatment may be considered in selected patients with less advanced cancer and less advanced IPF. Emphysema may occur in patients with IPF and is believed to represent a distinct clinical syndrome, known as the combined pulmonary fibrosis and emphysema (CPFE) syndrome. CPFE has a strong male predominance, is strongly linked with smoking, and has distinct radiographic features. CPFE is also associated with a very high frequency of pulmonary hypertension, which is associated with a poor prognosis. There are no specific treatments for CPFE and evaluation of IPF therapies in CPFE patients is urgently needed. (Sarcoidosis Vasc Diffuse Lung Dis 2015; 32; Suppl 1: 17-23)

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Published

03-08-2015

How to Cite

1.
Vancheri C, Cottin V, Kreuter M, Hilberg O. IPF, comorbidities and management implications. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2015 Aug. 3 [cited 2025 May 21];32(1S):17-23. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/4697