Idiopathic Interstitial Pneumonia

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Idiopathic Interstitial Pneumonia

  1. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral (often migratory) centrilobular ground glass nodules & Lower lung predominant peripheralground 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, & septalBronchiectasis/interlobular septal , surrounding consolidation Mild reticulation thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED Rarely evolve into lymphomaARDS (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, sarcoidhemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH
  2. 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, sarcoidAcute 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 30-40; M > F Age: 40-70; F > M Age: 20-55; M = FHigh mortality (50% < 2 months) Treatment: steroids Mortality 30% at 10 years Associations: Sjögren, AIDS Treatment: smoking cessationTreatment: ventilation/steroids Multiple basal predominant Treatment: smoking cessation Treatment: steroids subpleural/peribronchial Upper lung predominant faintDiffuse symmetric basal consolidations (often migratory) centrilobular ground glass Lower lung predominant Treatment in AIDS: anti-retroviralpredominant 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 perilymphaticBronchiectasis/interlobular septal Mild reticulation Mild reticulation nodules, & septal thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic pneumonia, NSIP, BAC, sarcoid, m SMOKING-RELATED SMOKING-RELATED Rarely evolve into lymphomaARDS (consolidation exceeds ultiple pulmonary DDx: DDx: DDx:ground glass), diffuse alveolar infarcts, lymphoma, lipoidhemorrhage, PAP, PCP, edema HP, DIP, pulm. LCH HP, RB-ILD, OP, NSIP, LIP, sarcoid HP, NSIP, DIP, pulmonary LCH pneumonia
  3. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviralground glass opacity (exceeds Lower lung predominant peripheral Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glassconsolidation) glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphaticBronchiectasis/interlobular septal Mild reticulation nodules, & septal thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphomaARDS (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, sarcoidPAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH
  4. 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 DADAge: pediatric to adult; M = F Age: 40-70; F > M Age: 50-60; M = F Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Associations: Sjögren, AIDS Treatment: steroids Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Treatment: steroids Multiple basal predominant Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant subpleural/peribronchial consolidations centrilobular ground glass nodules & Treatment in AIDS: anti-retroviralground 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, & septalBronchiectasis/interlobular septal Mild reticulation surrounding consolidation thickeningthickening may be present Air trapping Small (< 2 cm) well-defined cysts DDx: Small (< 3 cm) thin-walled cystsDDx: SMOKING-RELATED SMOKING-RELATED Chronic eosinophilic pneumonia, NSIP, Rarely evolve into lymphomaARDS (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, sarcoidhemorrhage, PAP, PCP, edema HP, NSIP, DIP, pulmonary LCH
  5. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviralground glass opacity (exceeds Lower lung predominant peripheral Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glassconsolidation) glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphaticBronchiectasis/interlobular septal Mild reticulation nodules, & septal thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphomaARDS (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, sarcoidPAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH
  6. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids subpleural/peribronchial Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant consolidations (often migratory) centrilobular ground glass nodules & Treatment in AIDS: anti-retroviralground glass opacity (exceeds Lower lung predominant peripheral Reverse halo sign: central ground patchy ground glass opacities subpleural ground glass attenuation Bilateral diffuse ground glassconsolidation) glass , surrounding consolidation Mild reticulation opacities, 2-4 mm perilymphaticBronchiectasis/interlobular septal Mild reticulation nodules, & septal thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED pneumonia, NSIP, BAC, sarcoid, multi Rarely evolve into lymphomaARDS (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, sarcoidPAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH
  7. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral (often migratory) centrilobular ground glass nodules & Lower lung predominant peripheralground 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, & septalBronchiectasis/interlobular septal , surrounding consolidation Mild reticulation thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED Rarely evolve into lymphomaARDS (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, sarcoidhemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH
  8. 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 DADAge: pediatric to adult; M = F Age: 50-60; M = F Age: 40-70; F > M Age: 20-55; M = F Age: 30-40; M > FHigh mortality (50% < 2 months) Treatment: steroids Associations: Sjögren, AIDS Treatment: smoking cessation Mortality 30% at 10 yearsTreatment: ventilation/steroids Multiple basal predominant Treatment: steroids Upper lung predominant faint Treatment: smoking cessationDiffuse symmetric basal predominant subpleural/peribronchial consolidations Treatment in AIDS: anti-retroviral (often migratory) centrilobular ground glass nodules & Lower lung predominant peripheralground 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, & septalBronchiectasis/interlobular septal , surrounding consolidation Mild reticulation thickeningthickening may be present DDx: Air trapping Small (< 2 cm) well-defined cysts Small (< 3 cm) thin-walled cystsDDx: Chronic eosinophilic SMOKING-RELATED SMOKING-RELATED Rarely evolve into lymphomaARDS (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, sarcoidhemorrhage, PAP, PCP, edema pneumonia HP, NSIP, DIP, pulmonary LCH

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