000 Idiopathic Pulmonary Fibrosis

  •  ILD
    • more than 200 lung diseases
      • UIPis the radiological correlate of IPF
    • idiopathic pulmonary fibrosis (IPF) is the most frequent
      • key radiololgy features
        • Subpleural and basal prese
        • traction bronchiolectasis
        • Others
          •  irregular reticulation,
            • fine reticulation
          • honeycombing,
            • clusters of cystic airspaces of
            • consistent diameter
            • multiple layers of
              • subpleural cysts
              • on top of each other
        • pleural thickening .  especially when associated with the presence of honeycomb cysts
        • Ground-glass opacification,
          •  not a typical feature of IPF
          • but can be seen in early disease.
      • Note
        • Traction bronchiectasis/ bronchiolectasis
          • and
        • honeycombing are  not always present,
        • making diagnosis of IPF difficult.
        • In addition,
          • UIP is also seen in other lung diseases
            • like hypersensitivity pneumonitis,
            • drug-induced lung disease and some
            • collagen vascular diseases
            • thus need for
              • multidisciplinary discussion.
    • Key Pathology features
      • predominantly subpleural
      • paraseptal  regions of
      • dense fibrosis with
      • yalinized collagen
        • destruction the alveolar architecture, and presents with
        • mild inflammatory infiltrate
      • honeycomb changes  characterized by formation of
        • cystic airspaces of varying size
        • filled with mucus, and
          • lined by bronchiolar epithelium
        •  myofibroblasts
          • adjacent to advanced fibrotic regions. I
          • Strata of Dx
            • A definite diagnosis
              • marked fibrosis with
              • architectural destruction,
              • patchy involvement and
              • fibroblast foci
          • Not UIP
            • presence of granulomas,
            • marked inflammation outside fibrotic regions,
            • hyaline membranes,
            • preferential airway-centered involvement and
            • organizing pneumonia,

 

References and Links

Attili, A.K etal  Smoking-related Interstitial Lung Disease: Radiologic-Clinical-Pathologic Correlation RadioGraphics Vol. 28, No. 5

Gupta et al Diffuse Cystic Lung Disease: Part I  American Journal of Respiratory and Critical Care Medicine 191(12)  April 2015

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