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20 Cards in this Set
- Front
- Back
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what are the characteristics of interstitial lung dz's (in general)
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thickened alveolar wall due to inflammation, edema and/or fibrosis
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what are the clinical signs/sx's of interstitial lung dz's?
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dyspnea, tachypnea, crackles, cyanosis (no wheezing)
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interstitial lung dz's will show OBSTRUCTIVE/RESTRICTIVE pattern on PFT.
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restrictive (remember it's the low lung volume and narrow curve)
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how do you diagnose ILD's?
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pretty tough, so may have to take lung biopsy if you really want to know etiology
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what is the typical pathogenesis of interstitial lung diseases?
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o Alveolitis – injury/inflammation of alveolar wall
o Organization – fibroblast proliferation o Fibrosis or resolution • Often results in “Honeycomb lung” – gross cystic spaces on biopsy |
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define idiopathic pulmonary fibrosis (aka. usual interstitial pneumonia).
tell me the pattern of fibrosis... |
characterized by chronic inflammation and fibrosis of the alveolar wall; begins with alveolitis, progreses to fibrosis, and ends in distorted fibrotic lung filled with cystic spaces (honeycomb) lung
HETEROGENOUS (patchy) FIBROSIS = varying fibroblastic foci and areas with different levels of severity and maturation |
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define acute interstitial pneumonia (Hamman-Rich Syndrome)
pattern of fibrosis? |
-rapid onset and progression with unknown cause
-Pathogenesis identical to DAD • Extensive involvement with interstitial inflammation, exudative and organizing alveolitis, and hyaline membranes HOMOGENOUS (uniform) FIBROSIS = all areas look identical |
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define non-specific interstitial pneumonia; what are the 2 types?
pattern of fibrosis? |
dz that mostly affects interstitium of alveolar walls;
2 types are CELLULAR PATTERN (mild/moderate infiltrates with lymphocytes/plasma cells only; no fibrosis) FIBROSING PATTERN – interstitial fibrosis with only mild lymphocytic infiltrates |
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what are the features absent in UIP?
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Hyaline membranes
> microfocus of organizing pneumonia Granulomatous inflammation Bronchiolocentric scarring dominant (UIP patchy/random) Marked interstitial inflammation (UIP only mild-mod) |
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what makes non-specific interstitial pneumonia important clinically?
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-better prognosis than UIP
- good resp to steroids |
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This type of interstitial lung disease is characterized by subpleural and peribronchial patchy airspace consolidation.
It has polyploid plugs of granulation tissue in bronchioles, alveolar ducts and alveoli. There is usually no interstitial fibrosis or honeycombing; may have interstitial lymphocytes and it recovers spontaneously or with steroids. dx? |
cryptogenic organizing pneumonia (which is basically an unknown cause for Bronchiolitis Obliterans Organizing Pneumonia - BOOP)
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what is the charactistic finding in sarcoidosis?
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non-caseating (non-necrotizing) granulomas
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what is hypersensitivity pneumonitis?
what histopathologic findings woudl you expect to see? |
type III hypersensitivity rxn to environmental antigens, like actinomyces (Farmer's lung)
o Poorly-formed granulomas (hard to see) with giant cells centered around airways o Bronchiolitis – inflammation around bronchioles o Mild interstitial lymphocytic infiltrates |
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what are the two smoking-related interstitial lung dz's?
what is the difference b/w the two? |
1) Respiratory Bronchiolitis-Associated Interstitial Lung Disease (RBILD)
- has airway centered increase in alveolar macrophages that contain "smoker's pigment) 2) Desquamative Interstitial Pneumonia (DIP) - also has alveolar pigmented macrophages, but JUST MORE...that's the big difference b/w these two. |
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this type of interstitial lung dz is characterized by dumbbell-shaped bodies with rounded ends and a straight core containing clear fibers
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asbestos bodies found in ASBESTOSIS
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coal miners are risk for what type of pneumoconioses (occupation-related lung dz)
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anthracosis
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define silicosis
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chronic occupational lung dz caused by exposure to free silica dust (seen in miners, glass manufacturers, stone cutters)
- dz initiated by ingestion of silica dust by alveolar macrophages; damage to macrophages initiates inflammatory resp mediated by lysosomal enzymes and various chem mediators - silicotic nodules enlarge and eventually obstruct airways and blood vessels |
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1. Which type of idiopathic interstitial pneumonia is characterized by temporal heterogeneity (multiple episodes of injury)?
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idiopathic pulmonary fibrosis
as opposed to acute intersitial pneumonia (which has a rapid course) |
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2. Name at least two types of ILD associated with granulomas.
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sarcoidosis (non-caseating granulomas), hypersensitivity pneumonitis (poorly formed granulomas)
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3. Compare and contrast the clinical, imaging, and pathologic features of asbestosis and silicosis.
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asbestosis will show up with chronic exposure to asbestos, whereas silicosis is due to chronic exposure to silica dust.
asbestosis will have asbestos bodies (dumbbell shaped) and cause diffuse mature interstitial fibrosis silicosis will show nodular fibrosis both silicosis and asbestosis will show mature, patchy fibrosis bc all pneumoconioses are like that |
