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1. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia (NSIP)
radiologic/pathologic finding; IPF is a
clinical diagnosis (of exclusion) based upon
UIP
Age: 40-70; M > F Age: 40-50; M = F
Median survival: < 3 yrs Mean survival: > 13 yrs
Treatment: supportive Treatment: steroids
Basal predominant Consider trial rx/open lung bx to confirm
Upper lung reticulation suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic NSIP, RA, chronic Temporospatial homogeneity
HP, asbestosis, sarcoid DDx:
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD) Lymphocytic Interstitial Pneumonia (LIP)
radiologic/pathologic finding; AIP is a (DIP)
clinical diagnosis (of exclusion) based upon
DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral
(often migratory) centrilobular ground glass nodules & Lower lung predominant peripheral
ground glass opacity (exceeds patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass opacities, 2-
consolidation) Reverse halo sign: central ground glass
Mild reticulation 4 mm perilymphatic nodules, & septal
Bronchiectasis/interlobular septal , surrounding consolidation Mild reticulation
thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
Rarely evolve into lymphoma
ARDS (consolidation exceeds ground pneumonia, NSIP, BAC, sarcoid, multiple DDx: DDx:
pulmonary infarcts, lymphoma, lipoid DDx:
glass), diffuse alveolar HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
hemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

2. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis
(IPF)
Nonspecific Interstitial
Usual Interstitial Pneumonia (UIP) Pneumonia (NSIP)
is a radiologic/pathologic finding;
IPF is a clinical diagnosis (of
exclusion) based upon UIP
Age: 40-70; M > F
Age: 40-50; M = F
Median survival: < 3 yrs
Mean survival: > 13 yrs
Treatment: supportive
Treatment: steroids
Basal predominant
Consider trial rx/open lung bx to
Upper lung reticulation confirm suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic Temporospatial homogeneity
NSIP, RA, chronic
DDx:
HP, asbestosis, sarcoid
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Lymphocytic Interstitial
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD)
radiologic/pathologic finding; AIP Pneumonia (DIP) Pneumonia (LIP)
is a clinical diagnosis (of exclusion)
based upon DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 30-40; M > F Age: 40-70; F > M
Age: 20-55; M = F
High mortality (50% < 2 months) Treatment: steroids Mortality 30% at 10 years Associations: Sjögren, AIDS
Treatment: smoking cessation
Treatment: ventilation/steroids Multiple basal predominant Treatment: smoking cessation Treatment: steroids
subpleural/peribronchial Upper lung predominant faint
Diffuse symmetric basal consolidations (often migratory) centrilobular ground glass Lower lung predominant Treatment in AIDS: anti-retroviral
predominant ground glass opacity nodules & patchy ground glass peripheral subpleural ground
Reverse halo sign: central ground opacities Bilateral diffuse ground glass
(exceeds consolidation) glass attenuation
glass , surrounding consolidation opacities, 2-4 mm perilymphatic
Bronchiectasis/interlobular septal Mild reticulation Mild reticulation nodules, & septal thickening
thickening may be present DDx:
Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic
pneumonia, NSIP, BAC, sarcoid, m SMOKING-RELATED SMOKING-RELATED Rarely evolve into lymphoma
ARDS (consolidation exceeds ultiple pulmonary DDx: DDx: DDx:
ground glass), diffuse alveolar infarcts, lymphoma, lipoid
hemorrhage, PAP, PCP, edema HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid HP, NSIP, DIP, pulmonary LCH
pneumonia

3. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia
radiologic/pathologic finding; IPF is a (NSIP)
clinical diagnosis (of exclusion) based
upon UIP
Age: 40-70; M > F
Age: 40-50; M = F
Median survival: < 3 yrs
Mean survival: > 13 yrs
Treatment: supportive
Treatment: steroids
Basal predominant
Consider trial rx/open lung bx to
Upper lung reticulation confirm suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic Temporospatial homogeneity
NSIP, RA, chronic
HP, asbestosis, sarcoid DDx:
CVD, drug rxn, IPF, DIP, OP, HP, PAP,
sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia Lymphocytic Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD)
radiologic/pathologic finding; AIP is a (DIP) (LIP)
clinical diagnosis (of exclusion) based
upon DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviral
ground glass opacity (exceeds Lower lung predominant peripheral
Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass
consolidation)
glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphatic
Bronchiectasis/interlobular septal Mild reticulation
nodules, & septal thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphoma
ARDS (consolidation exceeds ground DDx: DDx:
ple pulmonary DDx:
glass), diffuse alveolar hemorrhage, infarcts, lymphoma, lipoid HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

4. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia (NSIP)
radiologic/pathologic finding; IPF is a
clinical diagnosis (of exclusion) based upon
UIP
Age: 40-70; M > F Age: 40-50; M = F
Median survival: < 3 yrs Mean survival: > 13 yrs
Treatment: supportive Treatment: steroids
Basal predominant Consider trial rx/open lung bx to confirm
Upper lung reticulation suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic NSIP, RA, chronic Temporospatial homogeneity
HP, asbestosis, sarcoid DDx:
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD) Lymphocytic Interstitial Pneumonia (LIP)
radiologic/pathologic finding; AIP is a (DIP)
clinical diagnosis (of exclusion) based upon
DAD
Age: pediatric to adult; M = F Age: 40-70; F > M
Age: 50-60; M = F Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Associations: Sjögren, AIDS
Treatment: steroids Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Treatment: steroids
Multiple basal predominant Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant subpleural/peribronchial consolidations centrilobular ground glass nodules & Treatment in AIDS: anti-retroviral
ground glass opacity (exceeds Lower lung predominant peripheral
(often migratory) patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass opacities, 2-
consolidation)
Reverse halo sign: central ground glass , Mild reticulation 4 mm perilymphatic nodules, & septal
Bronchiectasis/interlobular septal Mild reticulation
surrounding consolidation thickening
thickening may be present Air trapping Small (< 2 cm) well-defined cysts
DDx: Small (< 3 cm) thin-walled cysts
DDx: SMOKING-RELATED SMOKING-RELATED
Chronic eosinophilic pneumonia, NSIP, Rarely evolve into lymphoma
ARDS (consolidation exceeds ground DDx: DDx:
BAC, sarcoid, multiple pulmonary infarcts, DDx:
glass), diffuse alveolar lymphoma, lipoid pneumonia HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
hemorrhage, PAP, PCP, edema HP, NSIP, DIP, pulmonary LCH

5. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia
radiologic/pathologic finding; IPF is a (NSIP)
clinical diagnosis (of exclusion) based
upon UIP
Age: 40-70; M > F Age: 40-50; M = F
Median survival: < 3 yrs Mean survival: > 13 yrs
Treatment: supportive Treatment: steroids
Basal predominant Consider trial rx/open lung bx to
Upper lung reticulation confirm suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic NSIP, RA, Temporospatial homogeneity
chronic HP, asbestosis, sarcoid DDx:
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia Lymphocytic Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD)
radiologic/pathologic finding; AIP is a (DIP) (LIP)
clinical diagnosis (of exclusion) based
upon DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviral
ground glass opacity (exceeds Lower lung predominant peripheral
Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass
consolidation)
glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphatic
Bronchiectasis/interlobular septal Mild reticulation
nodules, & septal thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphoma
ARDS (consolidation exceeds ground DDx: DDx:
ple pulmonary DDx:
glass), diffuse alveolar hemorrhage, infarcts, lymphoma, lipoid HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

6. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia
radiologic/pathologic finding; IPF is a (NSIP)
clinical diagnosis (of exclusion) based
upon UIP
Age: 40-70; M > F
Age: 40-50; M = F
Median survival: < 3 yrs
Mean survival: > 13 yrs
Treatment: supportive
Treatment: steroids
Basal predominant
Consider trial rx/open lung bx to
Upper lung reticulation confirm suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic Temporospatial homogeneity
NSIP, RA, chronic
HP, asbestosis, sarcoid DDx:
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia Lymphocytic Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD)
radiologic/pathologic finding; AIP is a (DIP) (LIP)
clinical diagnosis (of exclusion) based
upon DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviral
ground glass opacity (exceeds Lower lung predominant peripheral
Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass
consolidation)
glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphatic
Bronchiectasis/interlobular septal Mild reticulation
nodules, & septal thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphoma
ARDS (consolidation exceeds ground DDx: DDx:
ple pulmonary DDx:
glass), diffuse alveolar hemorrhage, infarcts, lymphoma, lipoid HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

7. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia (NSIP)
radiologic/pathologic finding; IPF is a
clinical diagnosis (of exclusion) based upon
UIP
Age: 40-70; M > F Age: 40-50; M = F
Median survival: < 3 yrs Mean survival: > 13 yrs
Treatment: supportive Treatment: steroids
Basal predominant Consider trial rx/open lung bx to confirm
Upper lung reticulation suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic NSIP, RA, chronic Temporospatial homogeneity
HP, asbestosis, sarcoid DDx:
CVD, drug rxn, IPF, DIP, OP, HP, PAP,
sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD) Lymphocytic Interstitial Pneumonia (LIP)
radiologic/pathologic finding; AIP is a (DIP)
clinical diagnosis (of exclusion) based upon
DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral
(often migratory) centrilobular ground glass nodules & Lower lung predominant peripheral
ground glass opacity (exceeds patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass opacities, 2-
consolidation) Reverse halo sign: central ground glass
Mild reticulation 4 mm perilymphatic nodules, & septal
Bronchiectasis/interlobular septal , surrounding consolidation Mild reticulation
thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
Rarely evolve into lymphoma
ARDS (consolidation exceeds ground pneumonia, NSIP, BAC, sarcoid, multiple DDx: DDx:
pulmonary infarcts, lymphoma, lipoid DDx:
glass), diffuse alveolar HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
hemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

8. Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis (IPF)
Usual Interstitial Pneumonia (UIP) is a Nonspecific Interstitial Pneumonia (NSIP)
radiologic/pathologic finding; IPF is a
clinical diagnosis (of exclusion) based upon
UIP
Age: 40-70; M > F Age: 40-50; M = F
Median survival: < 3 yrs Mean survival: > 13 yrs
Treatment: supportive Treatment: steroids
Basal predominant Consider trial rx/open lung bx to confirm
Upper lung reticulation suspected NSIP dx
Extensive subpleural disease Basal predominant
Honeycombing >> ground glass Upper lung sparing
Traction bronchiectasis Regions of subpleural sparing
Temporospatial heterogeneity Mixed ground glass/reticular
DDx: Traction bronchiectasis
CVD, drug rxn, fibrotic NSIP, RA, chronic Temporospatial homogeneity
HP, asbestosis, sarcoid DDx:
CVD, drug
rxn, IPF, DIP, OP, HP, PAP, sarcoid
Acute Interstitial Pneumonia (AIP)
Diffuse Alveolar Damage (DAD) is Desquamative Interstitial Pneumonia
Organizing Pneumonia (OP) Respiratory Bronchiolitis (RB-ILD) Lymphocytic Interstitial Pneumonia (LIP)
radiologic/pathologic finding; AIP is a (DIP)
clinical diagnosis (of exclusion) based upon
DAD
Age: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M
Age: 20-55; M = F Age: 30-40; M > F
High mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS
Treatment: smoking cessation Mortality 30% at 10 years
Treatment: ventilation/steroids Multiple basal predominant Treatment: steroids
Upper lung predominant faint Treatment: smoking cessation
Diffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral
(often migratory) centrilobular ground glass nodules & Lower lung predominant peripheral
ground glass opacity (exceeds patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glass opacities, 2-
consolidation) Reverse halo sign: central ground glass
Mild reticulation 4 mm perilymphatic nodules, & septal
Bronchiectasis/interlobular septal , surrounding consolidation Mild reticulation
thickening
thickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts
Small (< 3 cm) thin-walled cysts
DDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED
Rarely evolve into lymphoma
ARDS (consolidation exceeds ground pneumonia, NSIP, BAC, sarcoid, multiple DDx: DDx:
pulmonary infarcts, lymphoma, lipoid DDx:
glass), diffuse alveolar HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid
hemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH