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70 Cards in this Set
- Front
- Back
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What are the 4 main causes of Adult Respiratory Distress Syndrome?
Characterized by? |
1. Sepsis
2. Diffuse pulmonary infections/pneumonia 3. Gastric aspiration 4. Severe mechanical trauma with shock Rapidly progressive respiratory failure (24-48hrs), hypoxemia --> requires ventialtor |
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What is the histological manifestation of ARDS?
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Diffuse alveolar damage
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What is Hamman-Rich disease?
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idiopathic ARDS
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What is the pathogenesis of ARDS?
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Pro-inflammatory cytokines (IL8, IL1, TNF) released from macrophages
Chemotacitic to neutrophils, which release LT, PAF, Proteases --> 1. injure epithelial lining --> edema 2. induce hyaline membrane formation by leakage of proteins, prevents oxygen diffusion 3. necrosis of type II pneumocytes --> no more surfactant |
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What is the gross appearance of lungs in ArS?
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Lungs are diffusely heavy, dark red, airless
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What is the microscopic appearance of an ARDS lung?
2 stages? |
Diffuse alveolar damage
Acute stage: Intra-alveolar edema, hyaline membrane Organizing stage: proliferation of type II pneumocytes, progressive interstitial fibrosis |
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What is the clinical presentation of the exudative phase of ARDS?
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1. flu-like, progresses to severe dyspnea, hypoxemia
2. refractory to oxygen therapy (not helped) 3. profound hypoxia, requires ventilation |
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What is the clinical presentation of the organizing phase of ARDS (1 week)?
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Multi-organ failure
Mortality 60% patient can recover - chronic fibrosis |
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What are the characteristics of chronic restrictive diseases?
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fibrosis and inflammation of pulmonary interstitial tissue (alveolitis), plus decreased lung compliance
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What are three complications of chronic restrictive lung disease?
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Respiratory failure (honeycomb lung)
Pulmonary hypertension Cor Pulmonale |
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Restrictive lung diseases
a. symptoms b. imaging |
a. chronic dyspnea, hypoxia (cyanosis)
b. CXR: ground glass shadows, infiltrative/reticular pattern |
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What are 4 findings of Restrictive lung disease on Pulmonary function tests?
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1. Decreased TLC
2. Decreased Vital capacity 3. Absence of airflow obstruction 4. Decreased oxygen diffusion capacity |
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What is the end stage of all chronic restrictive lung diseases?
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diffuse interstitial fibrosis
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What is the general pathogenesis of chronic restrictive disease?
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Repeated cycles of acute injury and wound healing --> macrophages secrete IL8 --> neutrophils release mediates that injure epithelial cells, connective tissue, release TGFB for fibrosis
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What are 6 fibrosing chronic restrictive disease?
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IPF
nonspecific interstitial pneumonia cryptogenic organizing pneumonia pneumoconioses Collagen vascular disease Drugs |
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What are 2 granulomatous chronic restrictive diseases?
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Sarcoidosis
Hypersensitivity pneumonitis |
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What group of people is affected most by IPF?
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men, older than 60
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What is the gross morphology of IPF?
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cobblestone surface of lunge: dilated air spaces alternating with fibrous scars
interlobular septa: retraction of scar tissue Lower lobes, subpleural, interlobular septal regions: scarred, shrunken parenchyma |
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What is the gold standard for diagnosing IPF?
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Surgical lung biopsy
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What is the microscopic appearance of IPF?
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UIP pattern
Patchy interstitial fibrosis, accentuated subpleural Cystic spaces (honeycomb), interstitial lymphocytes |
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What does IPF look like on imaging?
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CT: Reticular strands, opacities towards periphery and base of lung
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Besides IPF, what other 2 restrictive lung disease have microscopic appearance of UIP (interstitial fibrosis, lymphocytes, honeycomb cystic spaces)
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Asbestosis
Rheumatoid lung disease |
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What is the clinical course of IPF?
Treatment? |
Dyspnea, dry cough, hypoxemia, respiratory failure
Lung transplantation |
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What causes honeycomb lung (pathologically)
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Remodeled airways (fibrosis and cysts) --> no longer available for gas exchange
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What are some morphological differences between honeycomb lung and lung disease caused by emphysema?
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Emphysema: No fibrosis, not nodular
Honeycomb: Patchy fibrotic parenchyma around cysts, nodular surface |
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What are 4 complications of IPF?
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Progressive fibrosis
End stage lung disease Pulmonary HT Acute exacerbations |
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How do nonspecific intersititial pneumonias differ from UIP?
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Treatable, diffuse
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What are the clinical features of NSIP?
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Dyspnea, cough
age 46-55 |
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What is the morphology of NSIP?
a. Microscopically b. Imaging |
a. Uniform interstitial inflammation and fibrosis, lymphocytic infiltrate
b. No honeycombing on CT |
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What are 3 conditions associated with NSIP?
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collagen vascular disease (SLE)
Drug rxn Chronic renal failure |
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What causes Cryptogenic organizing pneuonia (BOOP)?
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Idiopathic, post-infection, post-transplant, drugs, immunodeficiency sydndromes
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What is the cure for BOOP?
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Steroids for long periods
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What characterizes BOP?
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uniform polypoid masses of myxoid fibroelastic tissue in airways, alveoli
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What is pneumoconiosis?
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Lung reaction to inhalation of dusts and particulates (organic and inorganic)
esp. coal dust, silica, asbestos, berylium |
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What happens in coal workers pneumoconiosis?
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Accumulation of coal dust in lungs over 10 years --> uper lobe disease
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What are the three stages/severities of coal workers pneumoconiosis?
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1. Asymptomatic - carbon-laden macrophages accumulate in CT, no rxn
2. Simple: Carbon-laden macrophages, mild fibrosis --> emphysema 3. Complicated: increasing pulmonary dysfunction and fibrosis due to collagen and carbon-laden macrophages Fibrosis may persist after removal from exposure |
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What occurs in silicosis?
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Exposure to silica dust invades upper lobes, can increase susceptibility to TB
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What are the morphological signs of silicosis?
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Silicotic nodules (blue/green, upper zone) of hyalinized collagen with dust-laden macrophages
Central necrosis (superimposed TB) |
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What are 5 types of silicosis?
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1. Acute - large amount in short time
2. Nodular - fibrotic nodules (concentric hyalinization with macrophages in periphery) 3. Complicated: Eggshell calcification of hilar nodes 4. Silicotuberculosis - necrotizing granuloma 5. Rehumatoid pneumoconiosis |
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What are 5 clinical features of silicosis?
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1. asymptomatic with normal lung function tests
2. SOB develops late 3. increased TB 4. Caplan's - silicosis + rheumatoid lung disease |
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What are the 2 geometric forms of asbestosis?
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Serpentine - flexible, more prevalent
Amphibole - stiff, more pathogenic |
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What are 3 benign asbestos-related diseases?
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Pleural plaque/fibrosis (no effect on lung fn)
Pulm. interstitial fibrosis Pleural effusions |
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What are 3 malignant asbestos-related diseases?
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Bronchogenic cancer - smoking increases risk
Mesothelioma - smoking does NOT increase risk Laryngeal cancer |
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What is the morphology of asbestosis?
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Diffuse pulmonary fibrosis
Similar to UIP (lower lobes, subpleural) with asbestos bodies, can lead to honeycomb lung Often see pleural plaque (dense collagen in parietal pleura or diaphragm) |
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What are asbestos bodies?
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Fibers coated with iron-containing proteinaceous material
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Berylliosis
x ray appearance? Microscopic appearance? |
"butterfly" like sarcoidosis
non-necrotizing granulomas |
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What is progressive massive fibrosis of pneumoconiosis?
What 3 conditions have increased risk because of this? |
Massive fibrosis caused by particulates
TB, bronchitis, emphysema (NOT CANCER) |
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What is Caplan's Syndrome?
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Pneumoconiosis + Rheumatoid arthritis involving lung
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What histological features characterize sarcoidosis?
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non-caseating granulomas in lung with giant cells and lymphocytes
bilateral hilar lymphadenopathy Effects in eyes, skin, CNS, liver, heart |
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Who is more at risk for Sarcoidosis?
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younger adults (<40), women, african americans, non-smokers
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What are 3 important lab features of sarcoidosis?
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Increased ACE
Hypercalcemia (produces active vit D) Decreased CD4 lymphocytes |
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What are schaumann bodies?
asteroid bodies? |
schaumann - laminated concretions of Ca, proteins
astrocytes - eosinophilic stellate inclusions within giant cells SEEN in SARCOIDOSIS |
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How do the hilar nodes in sarcoidosis differ from those seen in TB?
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Sarcoidosis - enlarged
TB - matted, centrally necrosed, miliary spread |
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What is the pathogenesis of hypersensitivity pneumonitis?
3 types? |
Type III and IV hypersensitivity to organic dust antigens
Farmer's lung - actinomyces Pigeon breeder's lung Humidifier lung |
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What is the pathology of hypersensitivity pneumonitis?
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Patchy lymphcytic infiltrate, peribronchial accentuation and mild fibrosis
Non-caseating granulomas |
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How can you treat hypersensitivity pneumonitis?
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Remove source
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What are 2 smoking-related interstitial diseases?
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1. Desquamative interstitial pneumonia - pigmented macrophages in alveoli, mild interstitial fibrosis
2. Respiratory bronciolitis-associated interstitial lung disease - patchy fibrosis and macrophages with bronchocentric distribution |
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How do you treat smoking-related interstitial diseases?
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Stop smoking
Steroids |
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What are 5 pulmonary eosinophilic diseases?
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1. Acute eosinophilic pneumonia + respiratory failure
2. Simple pulmonary eosinophilia (Loffler) 3. Tropical 4. Secondary 5. Idiopathic chronic |
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What is the definition of Pulm HT?
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mean pulmonary bp reaches 1/4 of systemic level
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How can you treat hypersensitivity pneumonitis?
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Remove source
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What are 2 smoking-related interstitial diseases?
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1. Desquamative interstitial pneumonia - pigmented macrophages in alveoli, mild interstitial fibrosis
2. Respiratory bronciolitis-associated interstitial lung disease - patchy fibrosis and macrophages with bronchocentric distribution |
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How do you treat smoking-related interstitial diseases?
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Stop smoking
Steroids |
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What are 5 pulmonary eosinophilic diseases?
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1. Acute eosinophilic pneumonia + respiratory failure
2. Simple pulmonary eosinophilia (Loffler) 3. Tropical 4. Secondary 5. Idiopathic chronic |
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What is the definition of Pulm HT?
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mean pulmonary bp reaches 1/4 of systemic level
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What is the pathogenesis of Pulmonary hypertension?
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Small/med arterioles - medial hypertrophy, intimal proliferation
Large elastic -atheroma Severe pulm HT - plexogenic (tuft of capillary network in lumen of dilated small arteries) |
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What are diffuse alveolar hemorrhage syndromes? (3 symptoms)
3 examples? |
Immune-mediated diseases that present with hemoptysis, anemia, diffuse pulmonary infiltrates
1. Goodpastures 2. Idiopathic pulmonary hemosiderosis 3. Wegner's |
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What occurs in Goodpasture's?
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Anti-glomerular basement membrane Abs deposited linearly --> hemorrhagic interstitial pneumonitis, glomerulonephritis
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How id Idiopathic Pulmonary Hemosiderosis different from Goodpasture's?
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No renal involvement, no immunogloblin deposition
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What happensin wegner's granulomatosis?
How can you detect these |
Necrotizing vasculitis --> fever, bloody rhinorrhea, nasal ulcers, hemoptysis, hematuria, polyarthralgias
ANCA |
