ct scan of the chest in case Idiopathic Interstitial Pneumonias

Current List-ATS/ERS Consensus Classification

  • • Idiopathic Pulmonary Fibrosis (IPF) : Usual Interstitial Pneumonia (UIP)
  • • Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)
  • • Desquamative Interstitial Pneumonia (DIP)
  • • Acute Interstitial Pneumonia (AIP)
  • • Cryptogenic Organizing Pneumonia (COP)
  • • NonSpecific Interstitial Pneumonia (NSIP)

Idiopathic Pulmonary Fibrosis

  • Usual Interstitial Pneumonia: histologic pattern
  • 5th-7th decade : 66% > 60 years, 7/100,000 women and 10/100,000 men
  • Insidious onset of dyspnea : 6 months before diagnosis, Restrictive ventilatory defect, Rales and clubbing
  • Associations: Cigarette smoke, Dusty environments: farming, wood dust, metal dust, GE reflux, Autoantibodies common (ANA, RA)
  • Median survival 2.5-3.5 years

Usual Interstitial Pneumonia: Histology

  • Geographic variation
  • Temporal variation: Fibroblast foci, Mature fibrous tissue
  • Extensive fibrosis
  • Inflammation: Minimal, No correlation outcome
  • Abnormal wound healing
  • Prognosis : Fibroblast foci, Presence and extent

(Katzenstein, Am J Respir Crit Care Med 1998 Selman, Ann Int Med 2001King, Am J Respir Crit Care Med 2001)

Idiopathic Pulmonary Fibrosis Imaging:

  • • Radiograph abnormal-95%: Volume loss, Reticulonodular opacities, Lower lobe, Honeycombing
  • • Computed tomography : Peripheral and lower lobe, Reticulation and ground glass, Progress to honeycombing, Ground glass in areas of traction bronchiectasis

(Hartman, Chest 1996)

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Ct scan of the chest Typical peripheral reticulation and honeycombing and traction bronchiectasis in a patient with IPF

Utilility of Biopsy for Diagnosis of IPF

  • Prospective, multi-center study : 91 patients suspected of IPF
  • Clinical diagnosis : Positive predictive value with a confident diagnosis-87%
  • Imaging diagnosis: Positive predictive value with a confident diagnosis-96%, CT always abnormal in patients with proven IPF
  • Histologic diagnosis: Agreement regarding the presence or absence of IPF-85%, Agreement in patients without IPF-48%, Relevance to NSIP
  • Uncertain diagnosis
  • Discordant data
  • Disease other than IPF: Hypersensitivity pneumonitis, Collagen-vascular disease, Infection

(Hunninghake, Am J Respir Crit Care Med 2001)

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Chest X Ray Imaging