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)


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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