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