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

Question 1

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

Answer 1

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

Question 2

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

Answer 2

RV increases; FVC decreases

Question 3

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

Answer 3

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

Question 4

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

Answer 4

Cor pulmonale

Question 5

List four types of obstructive lung disease.

Answer 5

Chronic bronchitis, emphysema, asthma, and bronchiectasis

Question 6

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

Answer 6

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

Question 7

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

Answer 7

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

Question 8

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

Answer 8

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

Question 9

How is the Reid index calculated?

Answer 9

Reid index = gland layer thickness/total bronchial wall thickness

Question 10

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

Answer 10

Usually wheezing and crackles

Question 11

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

Answer 11

Cyanosis (early-onset hypoxemia from shunting)

Question 12

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

Answer 12

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

Question 13

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

Answer 13

Decrease, increase

Question 14

Name the two types of emphysema.

Answer 14

Centriacinar and panacinar

Question 15

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

Answer 15

Panacinar (this is emphysema)

Question 16

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

Answer 16

Elastase

Question 17

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

Answer 17

Airway pressure, airway collapse

Question 18

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

Answer 18

The bronchi

Question 19

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

Answer 19

Reversible

Question 20

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?

Answer 20

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

Question 21

What are Charcot-Leyden crystals?

Answer 21

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

Question 22

Name some triggers for bronchial hyperresponsiveness in asthmatics

Answer 22

Allergens, viral URIs, stress

Question 23

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

Answer 23

Test with methacholine challenge (the patient has asthma)

Question 24

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

Answer 24

Decreased inspiratory:expiratory ratio

Question 25

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

Answer 25

Pulsus paradoxus

Question 26

____ is a chronic necrotizing infection of the bronchi.

Answer 26

Bronchiectasis

Question 27

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

Answer 27

Permanent

Question 28

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

Answer 28

Blood and purulence

Question 29

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

Answer 29

Bronchiectasis

Question 30

Which two genetic diseases are associated with bronchiectasis?

Answer 30

Cystic fibrosis and Kartagener syndrome

Question 31

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

Answer 31

Allergic bronchopulmonary aspergillosis (he likely has bronchiectasis)

Question 32

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

Answer 32

Functional vital capacity and total lung capacity

Question 33

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

Answer 33

≥80%

Question 34

What are the two general types of restrictive lung disease?

Answer 34

Poor breathing mechanics and interstitial lung diseases

Question 35

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

Answer 35

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

Question 36

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

Answer 36

Normal; increased

Question 37

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

Answer 37

Polio

Question 38

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

Answer 38

Myasthenia gravis

Question 39

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

Answer 39

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

Question 40

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

Answer 40

Morbid obesity

Question 41

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

Answer 41

Interstitial lung diseases

Question 42

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

Answer 42

Decreased

Question 43

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

Answer 43

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

Question 44

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

Answer 44

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

Question 45

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

Answer 45

Sarcoidosis

Question 46

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

Answer 46

Restrictive lung disease (Goodpasture syndrome)

Question 47

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

Answer 47

Granulomatosis with polyangiitis (Wegener)

Question 48

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

Answer 48

Langerhans cell histiocytosis

Question 49

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

Answer 49

Hypersensitivity pneumonitis

Question 50

Name four drugs that can cause interstitial lung disease.

Answer 50

Bleomycin, busulfan, methotrexate, and amiodarone

Question 51

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

Answer 51

Bilateral hilar lymphadenopathy (this is likely sarcoidosis)

Question 52

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

Answer 52

80%

Question 53

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

Answer 53

Obstructive

Question 54

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

Answer 54

Obstructive

Question 55

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

Answer 55

Restrictive

Question 56

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

Answer 56

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

Question 57

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

Answer 57

In restrictive lung disease, both FEV1 and FVC are reduced

Question 58

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

Answer 58

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

Question 59

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

Answer 59

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

Question 60

What occupations are associated with exposure to asbestos?

Answer 60

Shipbuilding, roofing, and plumbing

Question 61

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

Answer 61

Lower, upper

Question 62

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

Answer 62

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

Question 63

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

Answer 63

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

Question 64

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

Answer 64

Upper lobes

Question 65

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

Answer 65

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

Question 66

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

Answer 66

Upper lobes

Question 67

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

Answer 67

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

Question 68

Patients with silicosis typically work in which three fields?

Answer 68

Foundries, sandblasting, mining

Question 69

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

Answer 69

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

Question 70

How does silicosis increase the risk of susceptibility to tuberculosis?

Answer 70

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

Question 71

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

Answer 71

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

Question 72

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

Answer 72

Bronchogenic carcinoma

Question 73

Which lobes of the lung are most affected by silicosis?

Answer 73

Upper lobes

Question 74

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

Answer 74

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