Resp - Path (Obstructive & Restrictive lung diseases)

Question 1

In general, what is the pathogenesis of obstructive lung disease?

Answer 1

Obstruction of air flow resulting in air trapping in the lungs. Airways close prematurely at high lung volumes –> high RV and low FVC. PFTs: very decreased FEV1, decreased FVC –> low FEV1/FVC ratio (hallmark). Chronic, hypoxic pulmonary vasoconstriction can lead to cor pulmonale.

Question 2

In general, how are lung volumes affected in obstructive lung disease?

Answer 2

Obstruction of air flow resulting in air trapping the lungs. Airways close prematurely at high lung volumes –> high RV and low FVC.

Question 3

In general, what are the PFT findings associated with obstructive lung disease? Which is its hallmark?

Answer 3

PFTs: very decreased FEV1, decreased FVC –> low FEV1/FVC ratio (hallmark).

Question 4

What is a complication of obstructive lung disease, and what causes it?

Answer 4

Chronic, hypoxic pulmonary vasoconstriction can lead to cor pulmonale

Question 5

What are the 2 main forms of COPD? What terms/nicknames help to distinguish them?

Answer 5

(1) Chronic bronchitis - “blue bloater” (2) Emphysema - “pink puffer,” barrel-shaped chest

Question 6

What type of disease is Chronic bronchitis? What is its pathogenesis?

Answer 6

A form of COPD along with emphysema; Hyperplasia of mucus-secreting glands in the bronchi –> Reid index (thickness of gland layer/total thickness of bronchial wall) > 50%

Question 7

Describe the cough of patients with Chronic bronchitis.

Answer 7

Productive cough for > 3 months per year (not necessarily consecutive) for > 2 years.

Question 8

What kind of airways are affected in Chronic bronchitis?

Answer 8

Disease of small airways

Question 9

What are 5 physical exam findings associated with Chronic bronchitis?

Answer 9

Findings: (1) wheezing (2) crackles (3) cyanosis (early-onset hypoxemia due to shunting) (4) late-onset dyspnea (5) CO2 retention

Question 10

What is the pathogenesis of Emphysema, and how does it affect the lung’s properties?

Answer 10

Enlargement of air spaces, decreased recoil, increased compliance, low DLCO resulting from destruction of alveolar walls

Question 11

What are the 2 types of Emphysema? With what is each associated?

Answer 11

Two types: (1) Centriacinar - associated with smoking (2) Panacinar - associated with alpha1-antitrypsin deficiency

Question 12

How does Emphysema affect compliance, and why?

Answer 12

Increase elastase activity –> loss of elastic fibers –> increased lung compliance

Question 13

What mechanism is used by Emphysema patients to prevent airway collapse during respiration?

Answer 13

Exhalation through pursed lips to increase airway pressure and prevent airway collapse during respiration

Question 14

What is asthma, and what effect does it have?

Answer 14

Bronchial hyperresponsiveness causes reversible bronchoconstriction.

Question 15

What are 3 histological findings associated with asthma?

Answer 15

(1) Smooth muscle hypertrophy (2) Curschmann spirals (shed epithelium forms mucus plugs) (3) Charcot-Leyden crystals (formed from breakdown of eosinophils in sputum)

Question 16

What are 3 triggers of asthma?

Answer 16

Can be triggered by viral URIs, allergens, and stress

Question 17

What is the test for asthma?

Answer 17

Test with methacholine challenge

Question 18

What are 8 physical/lab findings associated with asthma?

Answer 18

Findings: (1) cough (2) wheezing (3) tachypnea (4) dyspnea (5) hypoxemia (6) low I/E ratio (7) pulsus paradoxus (8) mucus plugging

Question 19

What causes bronchiectasis? What are its 4 main effects?

Answer 19

Chronic necrotizing infection of bronchi –> permanently dilated airways, purulent sputum, recurrent infections, hemoptysis

Question 20

What are 5 conditions/factors associated with bronchiectasis?

Answer 20

Associated with (1) bronchial obstruction (2) poor ciliary motility (smoking) (3) Kartagener syndrome (4) cystic fibrosis (5) allergic bronchopulmonary aspergillosis

Question 21

In general, what effect does restrictive lung disease have on lung volumes?

Answer 21

Restricted lung expansion causes decreased lung volumes (decreased FVC and TLC)

Question 22

What PFT finding is associated with restrictive lung disease?

Answer 22

PFTs: FEV1/FVC ratio >= 80%

Question 23

What are the 2 major types/mechanisms of Restrictive lung disease? Give a few characteristics that distinguish each of these major types.

Answer 23

(1) Poor breathing mechanics (extrapulmonary, peripheral hypoventilation, normal A-a gradient) (2) Interstitial lung diseases (pulmonary decreased diffusing capacity, increased A-a gradient)

Question 24

What are 2 ways that poor breathing mechanics can lead to restrictive lung disease? Give 2 specific examples of conditions for each of these ways.

Answer 24

(1) Poor muscular effort - polio, myasthenia gravis (2) Poor structural apparatus - scoliosis, morbid obesity

Question 25

What are 10 examples of interstitial disease types of restrictive lung disease?

Answer 25

(1) Acute respiratory distress syndrome (ARDS) (2) Neonatal respiratory distress syndrome (hyaline membrane disease) (3) Pneumoconioses (anthracosis, silicosis, asbestosis) (4) Sarcoidosis: bilateral hilar lymphadenopathy, noncaseating granuloma; high ACE and Ca2+ (5) Idiopathic pulmonary fibrosis (repeated cycles of lung injury and wound healing with increased collagen deposition) (6) Goodpasture syndrome (7) Granulomatosis with polyangitis (Wegener) (8) Langerhands cell histiocytosis (eosionophilic granuloma) (9) Hypersensitivity pneumonitis (10) Drug toxicity (bleomycin, busulfan, amiodarone, methotrexate)

Question 26

What is another name for neonatal respiratory distress syndrome? What type of restrictive lung disease does it cause?

Answer 26

Neonatal respiratory distress syndrome (Hyaline membrane disease); Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient)

Question 27

What are 3 examples of Pneumoconioses? What type of restrictive lung disease do they cause?

Answer 27

Pneumoconioses (anthracosis, silicosis, asbestosis); Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient)

Question 28

What type of restrictive lung disease does Sarcoidosis cause? What 2 physical/histological findings characterize Sarcoidosis? What are 2 substances that it increases?

Answer 28

Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient); Sarcoidosis: bilateral hilar lymphadenopathy, noncaseating granuloma; increased ACE and Ca2+

Question 29

What characterizes idiopathic pulmonary fibrosis? What type of restrictive lung disease does it cause?

Answer 29

Idiopathic pulmonary fibrosis (repeated cycles of lung injury and would healing with high collagen deposition); Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient)

Question 30

What type of granuloma is associated with Langerhans cell histiocytosis? What type of restrictive lung disease does it cause?

Answer 30

Langerhans cell histiocytosis (eosionophilic granuloma); Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient)

Question 31

What type of restrictive lung disease does drug toxicity cause? Give 4 examples of drugs that have this toxicity.

Answer 31

Interstitial lung disease (pulmonary decreased diffusing capacity, increased A-a gradient); Drug toxicity (bleomycin, busulfan, amiodarone, methotrexate)