Obstructive & Restrictive Lung Disease, Vascular Disease, Pleura Flashcards

(99 cards)

1
Q

3 types of acquired atelectasis

A

Resorption
Compression
Contraction

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2
Q

Atelectasis characterized by aiway obstruction with gradual resorption of air reducing lung expansion

A

Resporption

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3
Q

Atelectasis characterized by fibrotic or other innate restrictive process in the pleura or peripheral lung restricting lung expansion

A

Contraction atelectasis

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4
Q

Atelectasis characterized by accumulated material in pleural cavityy, squeezing the lung parenchyma

A

Compression atelectasis

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5
Q

Interstitial fluid in alveolar spaces shows up as pink proteinaceous material on histological slides of lung parenchyma, what condition is this indicative of?

A

Pulmonary edema

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6
Q

What are some hemodynamic causes of pulmonary edema?

A

“Pushing out” — Left sided heart failure (MCC), volume overload, pulmonary vein obstruction

“Leaking out” — hypoalbuminemia, nephrotic syndrome, liver dz

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7
Q

Pulmonary edema may also occur due to injury to alveolar wall. What might cause this type of injury?

A

Bacterial pneumonia
Sepsis
Smoke inhalation
Aspiration

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8
Q

What are 2 important causes of pulmonary edema of which the mechanism is unknown?

A

Neurogenic (brain injury)

High altitude

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9
Q

2 diseases on the ARDS spectrum

A

Acute lung injury (ALI) — acute onset, hypoxemia, bilateral infiltrates, no evidence of cardiac failure

Acute respiratory distress syndrome (ARDS) — worsening hypoxemia

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10
Q

Condition that is diagnosed based on presence of histologic manifestations of ARDS

A

Diffuse Alveolar Damage (DAD)

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11
Q

2 pathogenic mechanisms for infection with ARDS

A

Either due to inhalation or because something has entered bloodstream (sepsis, bacteremia, etc.) - so it can come from airway or circulation!

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12
Q

Physiologic measurement used to characterized degree of hypoxia in ARDS

A

PaO2/FiO2 ratio

If <300 it can be characterized as ALI, if it is <200 it can be characterized as ARDS [so ALI is basically mild form of ARDS]

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13
Q

4 characteristics necessary for diagnosis of ARDS

A

Abrupt onset of symptoms

Hypoxemia (PaO2/FiO2 <200)

Bilateral infiltrates

Non-cardiac in nature

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14
Q

Pathogenic mechanism of ARDS

A

Acute lung injury —> endothelial activation —> adhesion/extravasation of neutrophils —> accumulation of intraalveolar fluid, formation of hyaline membranes —> resolution of injury

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15
Q

Diffuse alveolar damage (DAD) is characterized by ______ membranes composed of edema + fibrin + cell debris.

What are 2 other characteristics associated with diffuse alveolar damage?

A

Hyaline

Decreased surfactant —> stiff lungs

Decreased aeration —> ventilation-perfusion mismatch (RBCs not getting close enough to airways due to hyaline!)

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16
Q

4 Stages of progression of ARDS

A
  1. Exudative (edema, hyaline membranes, neutrophils)
  2. Proliferative (fibroblast proliferation, organizing pneumonia, early fibrosis)
  3. Fibrotic (extensive fibrosis, loss of normal alveolar architecture)
  4. Either RESOLUTION or FIBROSIS —
    Resolution: restoration of normal cell structure/function
    Fibrosis: destruction/distortion of normal cell structure

NOTE fibrosis is IRREVERSIBLE

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17
Q

Compare/contrast acute interstitial pnuemonia different from ARDS/DAD?

A

AIP has same clinical presentation as ARDS

AIP has same histology as ARDS/DAD

Difference is that AIP cannot be attributed to a specific etiology (AIP often presents in middle aged women, usually preceded by a viral illness)

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18
Q

MCC of COPD/Chronic bronchitis

A

Smoking

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19
Q

Obstructive pulmonary disease characterized by alveolar wall destruction and overinflation

A

Emphysema

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20
Q

Obstructive pulmonary disease characterized by productive cough and airway inflammation

A

Chronic bronchitis

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21
Q

Reversible obstructive pulmonary disease characterized by bronchial hyperresponsiveness triggered by allergens, infection, etc.

A

Asthma

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22
Q

Diagnostic criteria for chronic bronchitis

A

Persistent cough with sputum production for 3 months out of 2 consecutive years

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23
Q

Predominant pathophysiologic mechanism in chronic bronchitis

A

Mucous gland hyperplasia —> damage to airway epithelium

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24
Q

3 primary complications of chronic bronchitis

A

Squamous metaplasia —> dysplasia —> carcinoma

Bronchiectasis

Death from respiratory infection

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25
Clinical presentation of emphysema on CXR and PFTs
CXR: Enlarged lungs, flattened diaphragm, and increased AP diameter PFTs: FEV1/FVC reduced
26
Characteristic patient presentation of chronic bronchitis
“Blue bloaters” Overweight and cyanotic Elevated hemoglobin Peripheral edema Rhonchi and wheezing
27
Characteristic patient presentation of emphysema
“Pink puffers” Older and thin Severe dyspnea Quiet chest X-ray: hyperinflation with flattened diaphragms
28
Pathogenic mechanism of alpha-1 antitrypsin deficiency
Deficiency in alpha 1 antitrypsin (trapped in liver, causes liver damage) leaves neutrophil elastase uninhibited, which causes lung damage
29
What type of emphysema is present in alpha1 antitrypsin deficiency?
Predominantly basilar panacinar emphysema [differentiate from COPD which is centrilobular, centriacinar, or proximal acinar]
30
Genetic mechanism of alpha 1 antitrypsin deficiency
Deficiency encoded by Pi (proteinase inhibitor) gene on Chr14; Z allele is associated with decreased circulating enzyme Homozygous PiZZ individuals have markedly decreased a1-AT, with a majority developing panacinar emphysema [Serum testing for a1-AT is the primary means of dx]
31
4 primary complications of emphysema
Respiratory failure Coronary artery disease Right heart failure (cor pulmonale) Pneumothorax with lung collapse
32
3 components of asthma
Recurrent airway obstruction with a reversible component Airway hyperresponsiveness Airway inflammation
33
What are the 2 types of asthma?
Atopic (extrinsic) — 2/3 of all pts Non-atopic (intrinsic) — 1/3 of all pts
34
Describe atopic asthma in terms of demographics and diagnostic features
Atopic asthma represents 2/3 of all asthma patients; may affect any age but typically presents in childhood There is usually family hx of asthma Characterized by elevated IgE levels (type I hypersensitivity) — eosinophils, mast cells, lymphocytes Triggers may include a variety of allergens
35
Describe non-atopic asthma in terms of demographics and diagnostic features
Non-atopic asthma represents 1/3 of all asthma pts, often older pts Typically normal IgE levels — T lymphocytes and neutrophils Triggers may include cold, exercise, infection
36
Immune mechanism of atopic asthma using pollen as example
Pollen Ag recognized by dendritic cell, which signals to Th2 CD4+ cell Th2 cell overreacts (hyperreactive phenomenon characteristic of asthma), and tells B cell lineage to form IgE antibodies at high levels Mast cells attracted by IgE, eosinophils recruited and the next time the immune system recognizes pollen, mediators react very strongly producing asthmatic symptoms
37
What mediators are responsible for the symptoms seen in asthma?
Bronchoconstriction is d/t Leukotrienes C4-D4-E4, histamine, prostaglandin D2, ACh Mucus secretion and increased vascular permeability d/t leukotriens C4-D4-E4 Recruitment of inflammatory cells due to interleukins
38
What are the consequences if asthma goes uncontrolled for long periods of time?
Progressive structural changes to airways with characteristic histologic findings = fibrosis, smooth muscle hyperplasia, increased goblet cells and submucosal glands Changes may be IRREVERSIBLE! There will also be a decreased response to therapeutic agents like bronchodilators and corticosteroids
39
Unremitting, possibly fatal asthma attack resulting from bronchial occlusion by thick mucus
Status asthmaticus
40
Characteristic histologic findings associated with status asthmaticus
Coiled mucus plugs: “Curshchmann spirals” Eosinophils and breakdown product — Charcot Leyden crystals
41
Other conditions and genetic factors associated with atopic asthma
Associated with seasonal allergies and eczema May be linked to various alleles controlling factors like IgE, cytokines (IL-4), adrenergic receptors, etc.
42
Environmental factors impacting asthma
Disease of industrialized societies — pollution, lack of allergen exposure at early age Early infection
43
Samter’s triad includes what specific type of asthma? What are the other 2 disease associations?
1. Aspirin-sensitive asthma 2. Nasal polyps 3. Recurrent rhinitis
44
Pathologic mechanism of aspirin sensitive asthma
When COX enzymes inhibited by aspirin or other NSAID, arachidonic acid is shunted into 5-lipoxygenase pathway which results in some production of leukotrienes C4-D4-E4, which are responsible for bronchoconstriction, mucus secretion, and increased vessel permeability resulting in asthmatic symptoms
45
End stage process of multiple chronic respiratory conditions (infection, obstruction, ABPA, CF, etc.) charcterized by a necrotizing inflammatory response
Bronchiectasis
46
What condition is defined by dysfunction of dynein arm of microtubules resulting in characteristic triad of respiratory symptoms as well as male infertility?
Kartagener’s syndrome: Primary Ciliary Dyskinesia
47
Triad of sx associated with Kartagener’s syndrome: Primary Ciliary Dyskinesia
Sinusitis Bronchiectasis Situs inversus
48
Difference in ciliary ultrastructure between PCD and CF
PCD has abnormal ciliary ultrastructure CF has normal ciliary ultrastructure; damage of cilia occurs secondary to other disease processes
49
Difference in etiology of male infertility in CF vs. PCD
CF — congenital bilateral absence of vas deferens PCD — immotile sperm (this disease results in abnormal cilia AND flagella!) [can also differentiate from Young’s syndrome male infertility which results from retained sperm in epididymis]
50
What is ABPA?
Allergic bronchopulmonary aspergillosis: exaggerated hypersensitivity response to aspergillus infection overlying chronic lung disease (asthma or cystic fibrosis)
51
ABPA results in increased ____ on serum testing, as well as positive _____ test. There is thick mucus in bronchi as well as ________ characteristic of aspergillus infection. It is associated with _______ in advanced disease
IgE; skin; fungal hyphae; bronchiectasis
52
Histologic findings with ABPA
Silver stain demonstrates aggregates of fungal hyphae characteristic of Aspergillus spp. (characteristic septations of hyphae and narrow angle branching)
53
5 categories of restrictive lung disease
Fibrosing: idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, connective tissue disease-associated, pneumoconiosis, drug reactions, radiation pneumonitis Granulomatous: sarcoidosis, hypersensitivity pneumonitis Eosinophilic Smoking related: desquamative interstitial pneumonia, respiratory bronchiolitis-associated ILD Other: Langerhans cell histiocytosis, pulmonary alveolar proteinosis, lymphoid interstitial pneumonia
54
Factors involved in pathogenesis of idiopathic pulmonary fibrosis
Environmental factors — industrialized societies, smoking Genetic factors Age — typically 50+
55
Clinical findings of idiopathic pulmonary fibrosis
Dyspnea Crackles on exam = “Velcro-like” Restrictive pattern on PFT’s — decrease in DLco, FVC Basilar infiltrates - progression to “honeycomb” lung
56
Classic findings on what test is required for diagnosis of idiopathic pulmonary fibrosis?
High-resolution CT scan OR pulmonary biopsy [biopsy shows usual interstitial pneumonia (UIP) characterized by normal and fibrotic areas, fibroblast foci, peripheral honeycombing]
57
T/F: Honeycomb fibrosis is specific to interstitial pulmonary fibrosis
False, it is just the end-stage of IPF
58
Prognosis of idiopathic pulmonary fibrosis
It is progressive, most pts expire from respiratory disease 3-5 years after diagnosis Potential therapies: lung transplant, medication to arrest fibrosis (tyrosine kinase inhibitors, TGF-beta inhibitors)
59
Idiopathic lung condition with unique histology consisting of uniform infiltrates and fibrosis, no heterogeneity, no fibroblast foci, no granulomata, and it has an overall better prognosis than UIP
Non-specific interstitial pneumonia (NSIP) [note that NSIP is NOT non-specific because it has unique histology]
60
Respiratory condition that presents with pneumonia-like consolidation in 5-6th decades and exists as a diagnosis of exclusion when there is no infection, drug or toxin, or relation to CT disorders
Cryptogenic Organizing Pneumonia (COP) [formerly BOOP — bronchiolitis-obliterans-organizing pneumonia]
61
Histologic findings and prognosis associated with cryptogenic organizing pneumonia
Fibroblast (Masson bodies) — organizing plugs of CT Good prognosis - pts tend to have full recovery with oral steroids
62
What autoimmune diseases can manifest as ILD (UIP, NSIP, organizing pneumonia, bronchioloitis)?
Rheumatoid arthritis Systemic sclerosis Lupus erythematosus [note that if ILD occurs as a manifestation of these conditions, they are not diagnosed as pure diseases, but the prognosis is linked to treatment of the underlying condition]
63
Of the interstitial lung diseases, which ones are considered granulomatous diseases?
Sarcoidosis | Hypersensitivity pneumonia
64
Systemic disease manifesting non-caseating (non-necrotizing) granulomata that presents as incidental abnormal radiograph, or with c/o dyspnea
Sarcoidosis
65
Clinical presentation of sarcoidosis
In addition to incidental radiograph findings or dyspnea, pts also tend to have: Elevated serum ACE levels Pts also tend to be <40 y/o, 10-fold predominance in african americans, commonly involves the lungs (also LNs or anywhere around the body) [etiology is likely immune-related; possible genetic predisposition]
66
Characteristic granuloma inclusions seen in sarcoidosis
Asteroid body Schaumann bodies [in addition to other granulomata findings like histiocytes, multinucleated giant cells, some lymphocytes, fibrotic sclerotic stroma, tight formation]
67
The stages of sarcoidosis do not occur in order. 20% of patients have progressive lung disease. Death may occur via involvement of what systems?
Pulmonary, cardiac, or neurologic
68
Respiratory condition caused by immune reaction to inhaled antigen resulting in airway-centered granulomata with associated lymphocytes
Hypersensitivity pneumonitis
69
Diagnosis of hypersensitivity pneumonitis requires a detailed history. What are some environmental exposures that might trigger this disease?
Pigeon breeder’s lung — protein from bird feces Farmer’s lung — actinomycetic spores in hay Hot tub lung — reaction to mycobacterium avium complex (MAC)
70
Disease affecting smokers in 4-5th decades with restrictive lung disease presentation and shows characteristic “stuffed” alveolar spaces full of macrophages on histology
Desquamative interstitial pneumonia (DSIP)
71
Treatment and prognosis of desquamative interstitial pneumonia (DSIP)
Treatment: stop smoking, corticosteroids Good prognosis: >95% survival at 5 years
72
Disease that exists as part of a spectrum with DSIP, but less symptomatic and earlier presentation (3rd-4th decades) and has dose-dependent relationship to smoking; radiographic abnormalities prompt biopsy for diagnosis
Respiratory brionchiolitis interstitial lung disease (RB-ILD)
73
Histologic findings associated with RB-ILD
Macrophages present to lesser extent “Peribronchiolar” metaplasia (abnormally located ciliated cells) May have fibrosis in advanced cases
74
T/F: RB-ILD is often reversible with smoking cessation if caught early
True
75
Lung disease found in young smokers and characterized by stellate lung lesions, progressive scarring leads to cysts and peripheral cysts may rupture and present with pneumothorax
Langerhans Cell Histiocytosis (LCH)
76
Histologic findings associated with Langerhans Cell Histiocytosis (LCH)
Eosinophils Langerhans cells (immature dendritic cells) — S-100 and CD1a positive!!! Varying fibrosis and cysts
77
What is the best way to reverse Langerhans Cell Histiocytosis?
Smoking cessation!
78
Respiratory disease characterized by impairment of surfactant metabolism due to defect of GM-CSF resulting in accumulation of surfactant proteins throughout alveoli and airspaces
Pulmonary alveolar proteinosis
79
Pulmonary alveolar proteinosis may be autoimmune (>90%), secondary to other disease, or hereditary (very rare). It can be treated with ______
Subcutaneous GM-CSF
80
Histologic and bronchioloalveolar lavage findings with pulmonary alveolar proteinosis
Histology: proteinaceous collections in alveolar spaces (appears similar to pulmonary edema on histology, need history to make dx) Fluid from bronchioalveolar lavage shows copious milky fluid
81
Reaction by the lungs to inhaled mineral or organic dust - often due to occupational exposure or air pollution (exaggerated response in some individuals indicates possible genetic component)
Pneumoconiosis
82
Aggravating factors of pneumoconiosis
Worse if exposure is high and repetitive, size of particles is small (means they can reach alveoli), and impaired ciliary clearance d/t smoking
83
Coal worker’s pneumoconiosis is a disease due to inhaled coal dust, with a spectrum of disease including what 3 components?
Anthracosis Coal macules/nodules Progressive massive fibrosis [most pts will not have progressive dz]
84
What is silicosis?
Disease resulting from inhaled silicon dioxide — occurs in mining/quarry work, concrete repair, demolition, etc. Insidious in onset, can occur after exposure is no longer present May progress to massive pulmonary fibrosis and has 2x risk of developing cancer (as opposed to CWP)
85
Histologic and radiographic findings characteristic of silicosis
Histology: dense collagenous nodules Radiographic: eggshell calcifications (calcified hilar LNs)
86
Interstitial and pleural disease resulting from inhalation of asbestos fibers in which risk correlates with exposure (insulation, shipyards, paper mills, oil or chemical refinery)
Asbestosis
87
Disease manifestations of asbestosis
Pleura — fibrosis/fibrous plaques, effusions, mesothelioma Lung — interstitial fibrosis, carcinoma Possibly extrapulmonary neoplasms
88
2 types of asbestos fibers and which one is most dangerous
Serpentine Amphibole — most dangerous, able to be inhaled deeper into lungs
89
Characteristic histologic finding associated with asbestosis
Asbestos bodies (consist of central filament, asbestos itself, and macrophage debris) Pleural plaque formation (known as “candlewax drippings”) manifests on histology as hyalinized collagen
90
Pulmonary emboli may be defined as obstruction of pulmonary vessels by what structures?
Blood clot (“true” thromboembolism) Bone marrow/fat Cancer (tumor embolus) Septic emboli Air Foreign material Parasites (dorofilaria)
91
In what patient populations might you see talc embolism?
IV drug users
92
Describe appearance of pulmonary infarcts
Wedge shaped lesions Begin as hemorrhagic, then fibrosis sets in (red—> white infarct)
93
Define pulmonary hypertension
Pulmonary artery pressure (PAP) greater than 25 mmHg
94
Histologic findings associated with pulmonary HTN
Plexiform lesion — very characteristic! Medial hypertrophy (smooth muscle in small arteries is good indicator)
95
3 pulmonary hemorrhage syndromes
Goodpasture syndrome Polyangiitis with granulomatosis (Wegener’s) Idiopathic pulmonary hemosiderosis
96
What does Goodpasture syndrome show on immunofluorescence?
Linear pattern of deposition d/t anti-basement membrane Abs
97
Pulmonary hemorrhage syndrome typically found in male smokers in their teens/20s, characterized by heavy red-brown lungs with necrosis, hemorrhage, and hemosiderin-laden macrophages, as well as linear deposits of Ig along basement membrane; clinical features include hemoptysis, focal pulmonary consolidation, renal failure, and possibly death d/t uremia
Goodpasture syndrome
98
Pulmonary hemorrhage syndrome d/t autoimmune dz associated with hemoptysis, capillaritis, and scattered, poorly formed granulomas unlike those seen in sarcoidosis
Polyangiitis with granulomatosis
99
Pulmonary hemorrhage syndrome associated with intermittent, diffuse alveolar hemorrhage of which the cause and pathogenesis is unknown; it is most often seen in young children, with insidious onset of productive cough, hemoptysis, and anemia, plus diffuse pulmonary infiltration
Idiopathic pulmonary hemosiderosis