Abstract


 
 
 
 Idiopathic pulmonary fibrosis (IPF) is defined as a chronic fibrosing interstitial disease of unknown cause, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP); it generally progresses into respiratory failure and death. Although progression of the disease is the most common cause of death, there are increasing reports of its association with other pathologies has been reported: e.g., IPF patients seem more susceptible to cardiovascular diseases. Therefore, other pathologies might also influence the natural course. In this paper, we describe a case of IPF and coronary artery disease (CAD). We emphasize the importance of cardiopulmonary exercise test (CPET) as a useful procedure to monitor disease progression in IPF patients. We also stress the importance of a careful analysis of variables measured for an accurate interpretation of the clinical picture and an improvement of the clinical management of patients. Moreover, we suggest that a careful assessment of CPET parameters may additionally help in the early detection of high cardiovascular ischemic risk.
 
 
 

Highlights

  • Idiopathic pulmonary fibrosis (IPF) is defined as a form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) [1]

  • IPF patients seem more susceptible to developing coronary artery disease (CAD) than people with non fibrotic lung disease or other non-IPF pulmonary fibrosis [5]

  • VCO2, carbon dioxide output; AT, anaerobic threshold), ventilatory/respiratory response (TV, tidal volume; Rf, respiratory frequency; VE, minute ventilation;VE/VCO2 and VE/VO2 ratios, ventilatory equivalents; VD/VT, ratio of physiologic dead space to tidal volume), and cardiovascular response (HR, heart rate; VO2/HR, oxygen-pulse; ΔVO2/ ΔWR, change in the oxygen uptake related to the increase in work rate), PaO2, PaCO2, pulmonary gas exchange

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Summary

Background

Idiopathic pulmonary fibrosis (IPF) is defined as a form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) [1]. Analysis of CPET, in spite of normal metabolic and ventilatory responses, showed an abnormal cardiovascular finding relative to the pattern of increased increase of increase VO2 relative to work rate below and above anaerobic threshold (Figure 1A): the ΔVO2/ΔWR ratio was reduced from 11.3 ml/min/W to 6.9 ml/min/; changes both in oxygen-pulse (Figure 1B) and in electrocardiographic signs that could be due to different causes were observed. IPF, by definition, is a progressive chronic fibrosing disease limited to the lung, the heart might be involved when gas exchange is so altered that coronary flow is affected In this context, CPET could be a useful tool to investigate subclinical CAD [10]. While the progression of the underlying lung disease is the most common cause of death for patients with IPF [2], various studies seem to suggest an additional increase in cardiovascular

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Raghu G
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