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Interstitial lung diseases
UIP
• a histologic pattern
• most common interstitial pneumonia, 40% of cases
• IPF accounts for about 70% of cases of UIP
Histology (hallmark is fibroblastic foci)
heterogeneous pattern (normal lung, interstitial fibrosis, fibroblastic
proliferation, honeycombing) ‘spatial heterogeneity’
abnormalities represent different stages of inflammation & fibrosis,
‘temporal heterogeneity’
Radiographs
• decreased lung volume is typical
• 80% - bilateral reticular pattern in the lower lung zones
• 10% - radiograph appears normal
• In earliest stages, fine reticulations in the posterior costo-phrenic
angles on lateral radiograph
• As fibrosis progresses, the reticular pattern becomes coarse, and
cystic areas of honeycombing are visible in about half of cases
CPFE
• IPF may be associated with emphysema
• characterized by upper lobe emphysema and lower lobe fibrosis
• frequently complicated by PHTN, acute lung injury, and lung cancer
• typically occurs in male smokers
• Mediastinal lymph node enlargement is visible in 70% of cases
(typically measure less than 15 mm)
• Patients may develop a fulminant and sometimes fatal acute
exacerbation characterized by consolidation or GGO; biopsy typically
shows DAD
NSIP
• accounts for 20% of IPs
• characterized histologically by alveolar wall thickening or fibrosis,
showing ‘spatial and temporal homogeneity’
all parts of the biopsy look the same and represent the same stage of
the disease
The diagnosis of NSIP should be considered if HRCT
shows ground-glass opacity with some degree of
reticulation, without honeycombing, in the sub-pleural
and basal lung, particularly if sub-pleural sparing is
present!!!
DIP & RB-ILD
• 10% of IPs
• characterized by the presence of intra-alveolar macrophages, mild
inflammation of alveolar walls, and minimal fibrosis.
• DIP - diffuse in distribution
• RB-ILD - bronchocentric (peribronchiolar) in distribution
strong association with smoking
• RB is a common incidental finding in asymptomatic smokers
• histologic finding of RB + pulmonary symptoms = RB-ILD
• DIP, RB, and RB-ILD represent different degrees of lung involvement
by the same process
HRCT features of fibrosis and progression of Abnormalities
HRCT
PATTERNS
Non Fibrotic HP
Fibrotic HP
NSIP
Organizing Pneumonia
Perilobular Consolidation
Unclassifiable ILDs
ILD
• Diverse group of conditions that may result in lung fibrosis
• Restrictive Pattern (low FVC, or low DLco)
Categorization of major Idiopathic Interstitial Pneumonias
Diagnostic Pointers
• Bronchocentric Pattern
• Subpleural Sparing
UIP BUT CTD
• Straight Edge Sign
• Anterior Upper lobe sign
• Exuberant Honey combing
sign
CPFE
Chest CT – Covid
• Typical
• Atypical
Typical Appearance of COVID 19
Indeterminate Appearance
Atypical Appearance
Post Covid Fibrosis
•
ILD presentation.pptx
ILD presentation.pptx

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ILD presentation.pptx

  • 2.
  • 3.
  • 4. UIP • a histologic pattern • most common interstitial pneumonia, 40% of cases • IPF accounts for about 70% of cases of UIP Histology (hallmark is fibroblastic foci) heterogeneous pattern (normal lung, interstitial fibrosis, fibroblastic proliferation, honeycombing) ‘spatial heterogeneity’ abnormalities represent different stages of inflammation & fibrosis, ‘temporal heterogeneity’
  • 5.
  • 6.
  • 7.
  • 8. Radiographs • decreased lung volume is typical • 80% - bilateral reticular pattern in the lower lung zones • 10% - radiograph appears normal • In earliest stages, fine reticulations in the posterior costo-phrenic angles on lateral radiograph • As fibrosis progresses, the reticular pattern becomes coarse, and cystic areas of honeycombing are visible in about half of cases
  • 9.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14.
  • 15. CPFE • IPF may be associated with emphysema • characterized by upper lobe emphysema and lower lobe fibrosis • frequently complicated by PHTN, acute lung injury, and lung cancer • typically occurs in male smokers
  • 16.
  • 17. • Mediastinal lymph node enlargement is visible in 70% of cases (typically measure less than 15 mm) • Patients may develop a fulminant and sometimes fatal acute exacerbation characterized by consolidation or GGO; biopsy typically shows DAD
  • 18.
  • 19. NSIP • accounts for 20% of IPs • characterized histologically by alveolar wall thickening or fibrosis, showing ‘spatial and temporal homogeneity’ all parts of the biopsy look the same and represent the same stage of the disease
  • 20.
  • 21.
  • 22.
  • 23.
  • 24.
  • 25. The diagnosis of NSIP should be considered if HRCT shows ground-glass opacity with some degree of reticulation, without honeycombing, in the sub-pleural and basal lung, particularly if sub-pleural sparing is present!!!
  • 26. DIP & RB-ILD • 10% of IPs • characterized by the presence of intra-alveolar macrophages, mild inflammation of alveolar walls, and minimal fibrosis. • DIP - diffuse in distribution • RB-ILD - bronchocentric (peribronchiolar) in distribution strong association with smoking
  • 27. • RB is a common incidental finding in asymptomatic smokers • histologic finding of RB + pulmonary symptoms = RB-ILD • DIP, RB, and RB-ILD represent different degrees of lung involvement by the same process
  • 28.
  • 29.
  • 30.
  • 31.
  • 32.
  • 33.
  • 34. HRCT features of fibrosis and progression of Abnormalities
  • 37.
  • 39.
  • 40. NSIP
  • 42.
  • 45.
  • 46. ILD • Diverse group of conditions that may result in lung fibrosis • Restrictive Pattern (low FVC, or low DLco)
  • 47. Categorization of major Idiopathic Interstitial Pneumonias
  • 48. Diagnostic Pointers • Bronchocentric Pattern • Subpleural Sparing
  • 49. UIP BUT CTD • Straight Edge Sign • Anterior Upper lobe sign • Exuberant Honey combing sign
  • 50.
  • 51. CPFE
  • 52. Chest CT – Covid • Typical • Atypical

Editor's Notes

  1. Architectural Distortion, Volume reduction
  2. Diagnosis of IPF , 2018
  3. Joint Recommendation of ATS ,ERS,
  4. Nomenclature. (Timeline is important )