5.Obstructive lung diseases-Restrictive lung disease-Obstructive vs. restrictive lung disease-Hypersensitivity pneumonitis-Pneumoconioses Flashcards

1
Q

What is the definition of obstructive lung disease? How does it affect lung volumes?

A

Obstruction to flow leading to air trapping & the collapse of airways at high volumes; RV increases due to air trapping, & FVC decreases

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2
Q

Does residual volume increase or decrease in obstructive lung disease? How about functional vital capacity?

A

RV increases; FVC decreases

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3
Q

What is the hallmark pulmonary function test finding in patients with obstructive lung disease?

A

Decreased ratio of FEV1 to FVC (the decrease in FEV1 > FVC)

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4
Q

A patient with chronic, hypoxic vasoconstriction can have what cardiac manifestation?

A

Cor pulmonale

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5
Q

List four types of obstructive lung disease.

A

Chronic bronchitis, emphysema, asthma, and bronchiectasis

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6
Q

A patient has a productive cough for 5 months over the course of 3 years. What findings do you expect on pulmonary function tests?

A

PFTs show a decreased ratio of FEV1 to FVC (the patient has classic chronic bronchitis)

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7
Q

A man has a productive cough for 5 months over 2 years with wheezing, crackles, cyanosis. What histologic changes are seen on lung biopsy?

A

Hyperplasia of the mucus-secreting glands in the bronchi (the patient has chronic bronchitis)

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8
Q

The mucus gland hyperplasia seen in chronic bronchitis can be quantified using the ____, which tends to be greater than what value (in %)?

A

Reid index (thickness of gland layer/total thickness of bronchial wall); >50%

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9
Q

How is the Reid index calculated?

A

Reid index = gland layer thickness/total bronchial wall thickness

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10
Q

A patient with chronic bronchitis presents to clinic. What clinical findings are auscultated in the lungs of this patient?

A

Usually wheezing and crackles

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11
Q

What visible skin finding may be noted in patients with chronic bronchitis?

A

Cyanosis (early-onset hypoxemia from shunting)

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12
Q

A woman has 6 months of productive cough in 3 years, wheezing, crackles, and cyanosis. What other findings would you expect in this patient?

A

• Expect late-onset dyspnea, secondary polycythemia, and hypercapnia (this patient has chronic bronchitis, as she is a “blue bloater”)

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13
Q

In emphysemic lungs, there is a(n) ____ (decrease/increase) in recoil and a(n) ____ (decrease/increase) in compliance.

A

Decrease, increase

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14
Q

Name the two types of emphysema.

A

Centriacinar and panacinar

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15
Q

A patient is diagnosed with α1-antitrypsin deficiency. What pattern of alveolar damage is this associated with?

A

Panacinar (this is emphysema)

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16
Q

In emphysema, the loss of elastic fibers and increased lung compliance result from increased activity of which enzyme?

A

Elastase

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17
Q

Individuals with emphysema tend to exhale through pursed lips to increase ____ and prevent ____ during expiration.

A

Airway pressure, airway collapse

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18
Q

In patients with asthma, there is hyperresponsiveness of what lung segment?

A

The bronchi

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19
Q

An important feature of the bronchoconstriction in asthma is that it is ____.

A

Reversible

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20
Q

A man with cough, tachypnea, and wheezing has a drop in BP >10 mmHg on inspiration. What pathologic lung findings do you expect on biopsy?

A

Curschmann spirals, smooth muscle hypertrophy, Charcot-Leyden crystals (this is asthma, which can exhibit pulsus paradoxus when severe)

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21
Q

What are Charcot-Leyden crystals?

A

Formed after the breakdown of eosinophils in sputum, they are eosinophilic, hexagonal, double-pointed needle-shaped crystals in asthmatics

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22
Q

Name some triggers for bronchial hyperresponsiveness in asthmatics

A

Allergens, viral URIs, stress

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23
Q

A child with cough, wheezing, dyspnea, and tachypnea has pulsus paradoxus on exam. What is a test for his condition?

A

Test with methacholine challenge (the patient has asthma)

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24
Q

What finding is commonly noted on pulmonary function tests of patients with asthma?

A

Decreased inspiratory:expiratory ratio

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25
Q

You measure the blood pressure of a patient having a severe asthma attack. What phenomenon might you observe with repeat measurements?

A

Pulsus paradoxus

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26
Q

____ is a chronic necrotizing infection of the bronchi.

A

Bronchiectasis

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27
Q

In bronchiectasis, chronic necrotizing infection of the bronchi leads to ____ (permanent/reversible) dilation of airways.

A

Permanent

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28
Q

A patient with bronchiectasis feels ill and has a productive cough. His sputum is most likely to consist of what?

A

Blood and purulence

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29
Q

Recurrent infections, bronchial obstruction, and poor ciliary motility may lead to what lung manifestation?

A

Bronchiectasis

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30
Q

Which two genetic diseases are associated with bronchiectasis?

A

Cystic fibrosis and Kartagener syndrome

31
Q

A patient has Kartagener syndrome leading to permanently dilated airways. Which fungal pulmonary infection is he prone to developing?

A

Allergic bronchopulmonary aspergillosis (he likely has bronchiectasis)

32
Q

What two lung volumes are typically decreased in patients with restrictive lung disease?

A

Functional vital capacity and total lung capacity

33
Q

Patients with restrictive lung disease typically have a FEV1/FVC ratio within what range?

A

≥80%

34
Q

What are the two general types of restrictive lung disease?

A

Poor breathing mechanics and interstitial lung diseases

35
Q

Extrapulmonary causes of restrictive lung disease are generally the result of what?

A

Poor breathing mechanics from muscular (e.g., polio, myasthenia gravis) or structural (e.g., scoliosis, morbid obesity) dysfunction

36
Q

Mechanical restrictive lung diseases have a(n) ___ A-a gradient. Interstitial lung diseases have a(n) ___ A-a gradient.

A

Normal; increased

37
Q

What infectious disease can cause poor muscular effort and lead to extrapulmonary restrictive lung disease and peripheral hypoventilation?

A

Polio

38
Q

What disease of the neuromuscular junction can cause poor muscular effort and thereby lead to extrapulmonary restrictive lung disease?

A

Myasthenia gravis

39
Q

A 32-y/o woman with neuromuscular disease, ptosis, diplopia, and muscle weakness at the day’s end may have what pulmonary manifestation?

A

Restrictive lung disease from poor muscular effort (the patient likely has myasthenia gravis)

40
Q

Other than scoliosis and muscle diseases, what condition can lead to extrapulmonary restrictive lung disease?

A

Morbid obesity

41
Q

Pulmonary causes of restrictive lung disease are generally the result of what category of diseases?

A

Interstitial lung diseases

42
Q

Restrictive lung disease due to acute respiratory distress syndrome (ARDS) would have ____ (increased/decreased) diffusing capacity.

A

Decreased

43
Q

A premature infant is diagnosed with hyaline membrane lung disease. What type of restrictive is this?

A

Interstitial type (this is neonatal respiratory distress syndrome [NRDS], also known as hyaline membrane disease)

44
Q

What category of interstitial lung diseases has a clear association with an environmental exposure?

A

Pneumoconioses (e.g., anthracosis, silicosis, asbestosis)

45
Q

A patient has bilateral hilar lymphadenopathy, noncaseating granulomas, and hypercalcemia. What interstitial lung disease does she have?

A

Sarcoidosis

46
Q

A patient has an autoimmune disease with hemoptysis and hematuria with renal failure. What kind of lung disease does this patient have?

A

Restrictive lung disease (Goodpasture syndrome)

47
Q

What granulomatous small-to-medium vessel vasculitis causes both glomerulonephritis and pulmonary dysfunction?

A

Granulomatosis with polyangiitis (Wegener)

48
Q

What disease can cause restrictive lung disease due to eosinophilic granulomas in the lungs?

A

Langerhans cell histiocytosis

49
Q

A patient has a cough and dyspnea at work that resolves when he leaves. What restrictive pulmonary process is likely causing his symptoms?

A

Hypersensitivity pneumonitis

50
Q

Name four drugs that can cause interstitial lung disease.

A

Bleomycin, busulfan, methotrexate, and amiodarone

51
Q

A patient has progressive dyspnea. Serum ACE and Ca2+ levels are elevated. What will a CXR show?

A

Bilateral hilar lymphadenopathy (this is likely sarcoidosis)

52
Q

In a normal, well-functioning lung, what percent does the FEV1:FVC ratio equal?

A

80%

53
Q

• In ____ (obstructive/restrictive) lung disease, FEV1 is more reduced than FVC.

A

Obstructive

54
Q

In ____ (obstructive/restrictive) lung disease, the FEV1:FVC ratio and lung volumes are increased.

A

Obstructive

55
Q

In ____ (obstructive/restrictive) pulmonary disease, the FEV1:FVC ratio and lung volumes are decreased.

A

Restrictive

56
Q

A COPD patient has PFTs performed. What would you expect his TLC, FRC, and RV to be compared to normal?

A

In obstructive lung disease, TLC, FRC, and RV are all increased

57
Q

A patient with silicosis has PFTs performed. What would you expect his FEV1 and FVC to be compared to normal?

A

In restrictive lung disease, both FEV1 and FVC are reduced

58
Q

A bird keeper develops dyspnea, cough, chest tightness, and a headache. What is the pathophysiology of his disorder?

A

A mixed III/IV hypersensitivity to environmental antigens (common in farmers and those around birds) (this is hypersensitivity pneumonitis)

59
Q

Silicosis, coal workers’ pneumoconiosis, and asbestosis are associated with increased risk of what two other diseases?

A

Cor pulmonale and Caplan syndrome (the combination of pneumoconiosis and rheumatoid arthritis)

60
Q

What occupations are associated with exposure to asbestos?

A

Shipbuilding, roofing, and plumbing

61
Q

Asbestosis mainly affects the ____(lower/upper) lung lobe(s), whereas silica and coal workers’ lung affects the ____(lower/upper) lobe(s).

A

Lower, upper

62
Q

A patient who works in an aerospace company is at risk for which type of pneumoconiosis?

A

Berylliosis, as working in the aerospace or manufacturing industries increases one’s risk for beryllium exposure

63
Q

A patient presents with shortness of breath and cough. He works for a local aerospace manufacturing plant. Treatment?

A

Steroids, as granulomas arise in berylliosis that may respond to this treatment

64
Q

Which lobes of the lungs are affected in a patient with berylliosis?

A

Upper lobes

65
Q

Explain the pathogenesis of coal workers’ pneumoconiosis (also known as black lung disease).

A

Prolonged exposure to coal dust results in carbon within macrophages, which leads to inflammation and fibrosis in the lungs

66
Q

Which lobes of the lungs are most affected by coal miners’ disease?

A

Upper lobes

67
Q

A patient is diagnosed with anthracosis. How does this differ from coal workers’ pneumoconiosis

A

Anthracosis is the result of exposure to sooty air in the city versus coal dust in coal workers (it is asymptomatic)

68
Q

Patients with silicosis typically work in which three fields?

A

Foundries, sandblasting, mining

69
Q

A foundry worker has dyspnea and cough. Chest X-ray shows calcification of his hilar lymph nodes. What cell type is responsible?

A

Macrophages respond to silica exposure by releasing fibrogenic factors, leading to fibrosis of the lungs (the patient has silicosis)

70
Q

How does silicosis increase the risk of susceptibility to tuberculosis?

A

Silica impairs macrophage phagolysosomes, affecting their ability to effectively kill microbes

71
Q

A sandblaster has egg-shell calcification on chest X-ray. What disorders is this patient at increased risk of developing?

A

This patient has silicosis, increasing risk for TB and bronchogenic carcinoma

72
Q

A foundry worker has dyspnea and cough. Chest X-ray shows hilar lymph node calcifications. He is at increased risk of what cancer type?

A

Bronchogenic carcinoma

73
Q

Which lobes of the lung are most affected by silicosis?

A

Upper lobes

74
Q

What is a mnemonic that helps you remember which pneumoconiosis affects the upper and lower lobes of the lungs?

A

Asbestos = from roof (insulation) but affects the base (lower lobes), silica and coal = from base (earth) but affect the roof (upper lobes)