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Towards a better diagnosis of idiopathic pulmonary fibrosis

D. Valeyre
European Respiratory Review 2011 20: 108-113; DOI: 10.1183/09059180.00001611
D. Valeyre
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  • For correspondence: Dominique.valeyre@avc.aphp.fr
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    Figure 1.

    Usual interstitial pneumonia pattern on high-resolution computed tomography (M. Brauner, Dept of Radiology, Hospital Avicenne, Bobigny, France; personal communication).

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    Figure 2.

    Usual interstitial pneumonia pattern on histopathology showing alternation of fibrosing areas and a) normal/subnormal parenchymal areas and b) fibroblastic foci (M. Kambouchner, Dept of Pathology, Hôpital Avicenne, Bobigny, France; personal communication).

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  • Table 1. Diagnosis of idiopathic pulmonary fibrosis based on patterns discovered in high-resolution computed tomography (HRCT) and histopathology investigations
    HRCT patternHistopathology pattern
    UIPProbable UIPPossible UIPNon-classifiable fibrosisNot UIP
    UIPYesYesYesYesNo
    Possible UIPYesYesProbableProbableNo
    Not UIPPossibleNoNoNoNo
    • UIP: usual interstitial pneumonia.

  • Table 2. Candidate biomarkers for idiopathic pulmonary fibrosis (IPF)
    KL-6A high-molecular weight glycoprotein highly expressed in tissue sections from patients with ILDs [35]. Elevated levels of serum KL-6 have been found in patients with clinically confirmed progression [33] and have been associated with increased mortality risk [36, 37].
    SP-A and SP-DC-type lectins produced mainly by alveolar epithelial type II cells. Serum levels of SP-A and SP-D are increased in IPF (but also other pulmonary diseases) [38–43] and are strongly predictive of mortality [44].
    CD28A co-stimulatory molecule normally expressed on most CD4+ T-cells. Down regulation of CD28 on peripheral CD4+ T-cells has been associated with increased risk of lung transplantation within 1 yr [45].
    Circulating fibrocytesThought to be progenitors for fibroblasts participating in the pathogenesis of lung fibrosis [46, 47]. Circulating fibrocytes were increased in IPF, with significant further increases during acute exacerbations. A proportion of >5% of peripheral blood leukocytes was associated with increased mortality in these patients [48].
    Angiogenic factorsThought to play a role in the pathogenesis of IIPs. Elevated levels of the potent angiogenic factors VEGF and IL-8 have been associated with IPF and progressive disease [49, 50].
    MMPsMatrix degrading enzymes thought to be critically involved in the pathology of pulmonary fibrosis [51–53]. Plasma MMP1 and MMP7 levels are significantly elevated in IPF patients [54]. MMP7 levels are consistently elevated in asymptomatic versus symptomatic IPF, indicating that it may be a marker for early disease [55].
    Oxidative stressMay be implicated in the epithelial dysfunction underlying pulmonary fibrosis [56]. Oxidant burden has been shown to be elevated in the serum [57] of IPF patients.
    • KL-6: Krebs von Lungen factor-6; SP: surfactant protein; MMP; matrix metalloproteinase; ILD: interstitial lung disease; IIP: idiopathic interstitial pneumonia; VEGF: vascular endothelial growth factor; IL: interleukin.

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Towards a better diagnosis of idiopathic pulmonary fibrosis
D. Valeyre
European Respiratory Review Jun 2011, 20 (120) 108-113; DOI: 10.1183/09059180.00001611

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Towards a better diagnosis of idiopathic pulmonary fibrosis
D. Valeyre
European Respiratory Review Jun 2011, 20 (120) 108-113; DOI: 10.1183/09059180.00001611
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  • Article
    • Abstract
    • DIAGNOSTIC CRITERIA FOR IPF
    • CHALLENGES IN DIAGNOSING IPF IN DAILY PRACTICE
    • THE NEED FOR EARLY DIAGNOSIS
    • IMPROVING DIAGNOSIS
    • CONCLUSION
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