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Predictors of shorter survival in patient with idiopathic pulmonary fibrosis

Predictors of shorter survival in patient with idiopathic pulmonary fibrosis
Clinical Morphologic Physiologic Molecular*
Demographic
  • Older age
  • Male sex

Symptoms

  • Worse dyspnea
  • Cough
  • Unintended weight loss

Physical exam

  • Lower body mass index

Comorbidities

  • Lung cancer
  • Cardiac disease
  • Pulmonary hypertension

Acute respiratory worsening

  • Acute exacerbation
  • Respiratory hospitalization
 High-resolution CT
  • Greater extent of fibrotic findings, ie, reticulation, traction bronchiectasis, and honeycombing (quantified by visual or automated methods)

Surgical lung biopsy

  • Greater profusion of fibroblast foci
 Pulmonary function tests
  • Lower FVC (especially <50%)
  • Lower DLCO (especially <35%)
  • 10% or greater decline in FVC over 3 to 12 months
  • 15% or greater decline in DLCO over 3 to 12 months
  • Composite physiologic index (composite score of FEV1, FVC, and DLCO)

Six-minute walk test

  • Shorter distance (especially <250 meters)
  • 50 meter or greater decline over six months
  • Desaturation
  • New onset hypoxemia
 Genomic
  • Shorter peripheral blood telomere length (especially <10th percentile for age)
  • Homozygous wild-type for MUC5B promotor polymorphism rs35705950
  • Hetero- or homozygous for TOLLIP minor allele rs5743890
  • Peripheral blood mononuclear cell gene expression profile

Blood proteins

  • Elevated levels of SPA or D, KL6, CCL18, MMP7, YKL40, Anti-HSP70 IgG, CXCL13, periostin, fibulin-1, collagen degradation products, CA 19-9, CA 125

Blood cells

  • Increased circulating fibrocytes

CT: computed cosmography; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; FEV1: forced expiratory volume in one second; MUC5B: mucin 5B; TOLLIP: toll interacting protein; SP: surfactant proteins; KL6: Krebs von den Lungen 6 (also called mucin 1); CCL18: CC chemokine ligand 18; MMP7: matrix metalloproteinase 7; YKL40: chitinase-3-like protein 1; HSP: heat shock protein; IgG: immunoglobulin g; CXCL13: C-X-C motif chemokine 13G; CA: cancer antigen.

* These molecular biomarkers are predictive of shorter survival in research studies, but are not ready for routine clinical use.
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