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40 Cards in this Set
- Front
- Back
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Diffuse Alveolar Hemorrhage
(Name the diseases) |
1. Goodpasture’s Syndrome
2. Idiopathic Pulmonary Hemosiderosis |
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What is the role of the radiologist in Non-neoplastic lesions?
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Development of high resolution CT
- Allowed better recognition and characterization of abnormalities than was possible with conventional chest radiography - Requires radiologist and pathologist to communicate well for clinicopathologic correlation |
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What is the role of the pathologist in Non-neoplastic lesions?
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Pattern recognition
Utilizes both GROSS and HISTOLOGY Viewed in two ways: 1. Anatomic distribution of pathologic lesions according to normal anatomic landmarks in the lung 2. Reaction patterns of the lung parenchyma |
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Usefulness of HRCT in the Evaluation of Lung Injury
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- allows for a specific or nearly specific dx in a small proportion of cases
- narrows considerably the ddx in many cases - is an aid in determining the biopsy site for the surgeon or bronchoscopist - aids the pathologist in narrowing the pathologic ddx - allows for a better assessment of the global extent and severity of dz than is possible pathologically based on a (relatively) small sample of lung tissue |
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Pattern Information gained by HRCT
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- Distribution of lesions in the lungs as a whole (upper lobe vs. lower lobe, central vs. peripheral)
- Predilection for a specific anatomic region (centrilobular vs. septal) - Qualitative character of the abnormalities identified (ground glass vs. consolidation) - quantitative extent of the abnormalities by visual estimate |
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Pathologist responsibility
in ddx |
Focused on separating the type of histologic pattern
- Is it a UIP, organizing pneumonia, or DAD pattern? Be concerned about potential etiologies - Drug reaction, environmental or occupational exposures, or associated connective tissue disease. (Example: Bronchiolocentric cellular interstitial pneumonia pattern with granulomas means that the clinician should go back and ask about exposure to inhaled antigens, drugs, or toxic substances (communication is crucial)) |
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Clinician responsibility
in ddx |
To address most of the etiologic possibilities
Ultimately determine whether process is idiopathic or not (not uncommon) |
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ill-formed granulomas suggesting
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a hypersensitivity pneumonitis pattern
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Asbestos bodies suggestive of
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asbestosis
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Giant cell interstitial pneumonia suggestive of
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hard metal pneumoconiosis
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Diffuse Alveolar Damage (DAD)
Clinical Presentation |
1. Acute onset of dyspnea
2. Diffuse pulmonary infiltrates 3. Rapid respiratory failure 4. Multiple underlying causes |
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Diffuse Alveolar Damage (DAD)
Pathologic Pattern |
Correlation with clinically label of “acute respiratory distress syndrome (ARDS)”
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Diffuse Alveolar Damage (DAD)
Idiopathic Variant |
Known as Acute Interstitial Pneumonia (AIP)
- Hamman-Rich Syndrome |
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Causes of Diffuse Alveolar Damage
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Shock
Infection (severe bacterial pneumonia) Trauma Aspiration of Gastric Contents Inhalation injury Drugs Radiation Metabolic Disorders Hematologic Disorders Idiopathic and many others... |
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DAD
Pathologic Features |
Temporally uniform injury
Occurs in two phases: 1. Exudative stage (acute) 2. Organizing stage (proliferative) |
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Exudative Stage
DAD Pathologic Features |
1. Interstitial edema
2. Type I pneumocyte sloughing 3. HYALINE MEMBRANES |
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Organizing Stage
DAD Pathologic Features |
1. Proliferating type II pneumocytes
2, Interstitial fibroblasts 3. (focal airspace organization) |
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CT of
Early Exudative Stage of ARDS |
Demonstrates patchy bilateral areas of GROUND GLASS attenuation and consolidation
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CT of
Late Exudative Phase of ARDS |
- Demonstrates areas of consolidation in dependent lung regions and extensive areas of ground-glass attenuation
- Note focal areas of relatively normal parenchyma |
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What does ground-glass on CT mean?
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diffuse
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DAD Exudative Stage
Microscopy |
Type 1 pneumocytes and endothelial cells:
--> Edema and exudation of plasma proteins into alveolar interstitium and spaces Type 1 pneumocytes: - Undergo extensive necrosis - Slough off alveolar surface Underlying Basement Membrane: - Denudation - Becomes surface of attachment for hyaline membranes and fibrin Eosinophilic “membranes”: - Line alveolar surfaces - Consist of precipitated plasma proteins and cytoplasmic and nuclear debris from sloughed epithelial cells |
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DAD Organizing Stage
Microscopy |
Proliferation of loose, organizing type of connective tissue (early fibrosis) in the alveolar septal walls
Prominent lining of HYPERPLASTIC TYPE 2 PNEUMOCYTES |
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The new name for
Bronchiolitis Obliterans Organizing Pneumonia (BOOP) |
Cryptogenic Organizing Pneumonia (COP)
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Clinical Presentation of BOOP/COP
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Subacute onset:
- Cough - Dyspnea - Fever |
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BOOP/COP
CXR |
Multiple patchy airspace opacities on CXR
Usually bilateral |
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What tx is there for BOOP/COP?
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Favorable course with corticosteroids
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Causes of BOOP/COP
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Idiopathic
Infectious agents: - Viral - Bacterial Inhalational injury Autoimmune disorders/collagen vascular disease Aspiration Nonspecific reaction adjacent to other lesions in the lung (e.g., abscess, infarct or NEOPLASM) |
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BOOP/COP
Key Histologic Features Pertinent Positives |
Patchy distribution
Intraluminal loose/immature connective tissue (organizing fibrosis) in distal airspaces (esp. alveolar ducts) Preservation of lung architecture Temporally uniform appearance |
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BOOP/COP
Histologic Features Pertinent Negatives |
Minimal or lack of interstitial connective tissue/fibroblasts
Lack of hyaline membranes |
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Name the Diffuse Alveolar Hemorrhage Diseases
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Goodpasture’s Syndrome
Idiopathic Pulmonary Hemosiderosis |
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Goodpasture’s Syndrome
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M:F = 9:1
>> in young adults Smokers HLA DRw15, DQw6 Antibody-mediated immune reaction to basement membranes (IgG) in both LUNG and KIDNEY |
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Idiopathic Pulmonary Hemosiderosis
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M:F = 1:1
>> in adolescents and children - 20% are adults <30 Hemoptysis & chronic hemorrhage - Sometimes with acute hemorrhage No renal involvement Etiology unknown - No immunological mechanism |
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When do you see hemoptysis with Goodpasture's Syndrome?
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Acute and/or chronic hemorrhage
Anemia Renal insufficiency Diffuse lung infiltrates |
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How do we visualize alveolar septa in Goodpasture's Syndrome?
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Direct immunofluorescence shows linear IgG staining of alveolar septa
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What besides Goodpasture's Syndrome and Idiopathic Pulmonary Hemosiderosis can cause ACUTE HEMORRHAGE?
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Necrotizing Capillaritis
Churg-Strauss |
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With what can Pulmonary Alveolar Proteinosis be confused?
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pneumocystis
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With what is Pulmonary Alveolar Proteinosis associated?
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1. Autoimmunity
2. Dust 3. Drugs 4. Infection 5. Leukemia/lymphoma 6. idiopathic |
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Pulmonary Alveolar Proteinosis
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Predominantly affects adults ages 30-50
Non-productive/productive cough with expectoration of gelatinous material |
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Pulmonary Alveolar Proteinosis
Mutant (knockout) mice |
Lacking gene for granulocyte-macrophage-colony-stimulating-factor (GM-CSF)
Similar accumulation of surfactant and surfactant apoprotein in alveolar spaces Reconstitution of respiratory epithelium with GM-CSF gene - Completely corrects the alveolar proteinosis |
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Pulmonary Alveolar Proteinosis
Mechanism |
Due to IMPAIRED CLEARANCE OF ALVEOLAR SURFACTANT by alveolar macrophages
Studies demonstrate defect in alveolar macrophage function (impaired ability to process surfactant) play significant role in pathogenesis **IgG antibodies against GM-CSF are present in patients with primary acquired pulmonary alveolar proteinosis |
