First Aid: Obstructive and restrictive lung disease

Question 1

What happens to the following lung volumes in obstructive and restrictive lung disease?

a) RV
b) FRC
c) TLC
d) FEV1
e) FVC
f) FEV1/FVC

Answer 1

Obstructive:
Increased: RV, FRC and TLC
Decreased: FEV1 (very decreased), FVC and FEV1/FVC

Restrictive:
Decreased: RV, FRC, TLC, FEV1, FVC
Increased or normal: FEV1/FVC as the FEV 1 has decreased proportionately with the FVC

Question 2

How is the flow-volume loop changed in obstructive and restrictive lung disease?

Answer 2

Obstructive: shifts left
Restrictive: Shifts right (R-R)

Question 3

What two diseases comprise COPD?

Answer 3

Chronic bronchitis and emphysema

Question 4

What is the pathology of obstructive lung disease?

Answer 4

Obstructed airflow leads to increased trapping of air in the lungs. Airways close prematurely at high lung volumes, leading to further trapping

Question 5

What can chronic hypoxic pulmonary vasoconstriction lead to?

Answer 5

Cor pulmonale

Question 6

How does chronic bronchitis commonly present?

Answer 6

Blue Bloater: WCPCDC

Wheezing, cyanosis, can cause secondary polycythemia, CO2 retention, dyspnea, crackles

Question 7

How does chronic bronchitis cause secondary polycythemia?

Answer 7

As it leads to chronic hypoxemia which triggers increased EPO from the kidneys

Question 8

What is the pathology of chronic bronchitis?

Answer 8

Hyperplasia and hypertrophy of the mucus-secreting glands

Question 9

What is the Reid index and what would it indicate in chronic bronchitis?

Answer 9

Pathological measurement of the ratio between the thickness of submucosal mucus-secreting glands and the thickness between the cartilage and epithelia that line the bronchi, in chronic bronchitis this would be >50%

Question 10

What is the diagnostic criteria for chronic bronchitis?

Answer 10

Productive cough for >3 months in a year, for two consecutive years

Question 11

How does emphysema commonly present?

Answer 11

Barrel chest and pursed lips on expiration (to increase airway pressure and prevent airway collapse)

Question 12

Describe the two pathological patterns of emphysema, what they’re associated with and the lobes involved

Answer 12

Centriacinar: affects the upper lobes, respiratory bronchioles and proximal alveoli (with sparing of the distal alveoli). Associated with smokers

Panacinar: affects the whole lung but more commonly the lung base, (including respiratory bronchioles -> distal alveoli), associated with alpha1 antitrypsin deficiency

Question 13

Why is there an increase in lung compliance in emphysema?

Answer 13

An imbalance of proteases and antiproteases leads to increased elastase, this decreases the elastic recoil fibres and increases lung compliance

Question 14

What happens as a result of the enlargement of air spaces in emphysema?

Answer 14

Decreased: recoil, DLCO (from the destruction of alveolar walls) and blood volume in the pulmonary capillaries

Increased: compliance

Question 15

What is found on a CXR in emphysema?

Answer 15

1. Increased AP diameter
2. Increased lung field lucency
3. flattened diaphragm

Question 16

What are the clinical findings of asthma?

Answer 16

DWCTHIMP: dyspnea, wheeze, cough, tachypnea, hypoxemia, decreased inspiratory/expiratory ratio, mucus plugging, pulsus paradoxus

Question 17

What are the triggers for asthma?

Answer 17

Viral URIs, allergens and stress

Question 18

What is pulsus paradoxus and what causes it?

Answer 18

A significant drop in BP during inspiration (>10 mmHg)

RILE: increased bloodflow to R heart on inspiration and increased bloodflow to L heart on expiration. When the patient inspires the increase in blood in the R heart pushes on the septum, decreasing the space for blood to fill the L heart -> resulting in a decreased CO and BP

Question 19

Describe the pathology of asthma (including the two microscopic findings)

Answer 19

1. Hyper responsive bronchi leading to reversible bronchoconstriction
2. Hyperplasia and hypertrophy of the smooth muscle cells

Microscopic findings:

3. Curschmann spirals: mucus spiral plugs from subepithelial mucus gland ducts of bronchi
4. Charcot-Leyden crystals: hexagonal, double-pointed eosinophilic crystals formed via eosinophilic degradation in the sputum

Question 20

What type of hypersensitivity reaction occurs in asthma?

Answer 20

Type 1

Question 21

What supports the diagnosis of asthma?

Answer 21

Spirometry and the methacholine challenge

Question 22

What makes up NSAID exacerbated respiratory disease?

Answer 22

COX inhibitors (inhibit cyclooxygenase (COX) which helps produce prostaglandins from arachidonic acid, with this inhibited arachidonic acid forms leukotrienes (which are released from mast cells during an asthma attack) and cause bronchoconstriction

Chronic sinusitis w nasal polyps (a result of chronic inflammation)

Asthma symptoms

Question 23

What are the clinical findings in bronchiectasis?

Answer 23

PPHD: purulent sputum, recurrent infections (most commonly P. aeruginosa), hemoptysis and digital clubbing

Question 24

What happens pathologically in bronchiectasis and why?

Answer 24

Permanently dilated airways due to chronic necrotizing infection of the bronchi or obstruction

Question 25

What is bronchiectasis associated with?

Answer 25

ABCC 1. Allergic bronchopulmonary aspergillosis 2. Bronchial obstruction 3. CF 4. Impairment of mucociliary escalator: i.e due to smoking or kartagener syndrome (rare autosomal recessive ciliary disorder)

Question 26

What are some associated conditions when there is poor breathing mechanics in restrictive lung disease? Is it extrapulmonary or pulmonary, and how does it affect the DLCO and A-a gradient?

Answer 26

1. Poor muscular effort: polio, MS, Guillain barre 2. Poor structural apparatus: scoliosis, morbid obesity Extrapulmonary, normal DLCO and A-a gradient

Question 27

What are some associated conditions when there is 'interstitial disease' in restrictive lung disease? Is it extrapulmonary or pulmonary, and how does it affect the DLCO and A-a gradient?

Answer 27

GLISPHD 1. Granulomatosis with polyangiitis (Wegener's granulomatosis) 2. Pulmonary Langerhans cell histiocytosis (eosinophilic granuloma) 3. Idiopathic pulmonary fibrosis 4. Sarcoidosis 5. Pneumoconioses (i.e coal workers pneumoconiosis, silicosis, asbestosis) 6. Hypersensitivity pneumonitis 7. Drug toxicity (i.e methotrexate, bleomycin, busulfan, amiodarone) Pulmonary, decreased DLCO and increased A-a gradient

Question 28

What happens in idiopathic pulmonary fibrosis? How does the lung appear?

Answer 28

When there are repeated cycles of lung injury and wound healing with increased collagen deposition, the lung has a "honeycomb" appearance

Question 29

What is hypersensitivity pneumonitis? What symptoms can it cause, and where is it commonly seen? Is it reversible?

Answer 29

A mixed type 3/4 hypersensitivity reaction to an environmental antigen, can cause: dyspnea, cough, chest tightness, fever and headache Commonly seen in farmers and those exposed to birds and is reversible in early stages if the stimulus is avoided

Question 30

What is sarcoidosis characterized by? Describe what you would find in the serum levels, bronchoalveolar lavage fluid, on a CXR and a CT scan

Answer 30

Characterized by immune-mediated widespread noncaseating granulomas Serum: elevated ACE and Ca2+ (hypercalcemia is due to the increase in 1alpha hydroxylase-mediated vitamin D activation in macrophages) Bronchoalveolar lavage fluid: elevated CD4/CD8 ratio CXR: bilateral adenopathy and coarse reticular opacities CT: better demonstrates the extensive hilar and mediastinal adenopathy

Question 31

What population is most commonly affected by sarcoidosis? Is it symptomatic?

Answer 31

African American females, often asymptomatic other than enlarged lymph nodes

Question 32

What conditions are associated with sarcoidosis?

Answer 32

A facial droop is UGLIER (neurosarcoidosis can cause sudden facial weakness) U: Uveitis G: granulomas (noncaseating epithelioid, containing microscopic Schaumann and asteroid bodies) L: Lupus pernio (skin lesions on face resembling lupus; hard and purplish) I: Interstitial fibrosis E: erythema nodosum R: rheumatoid arthritis-like arthropathy

Question 33

How is sarcoidosis treated?

Answer 33

With steroids, if it's symptomatic

Question 34

What causes inhalation injury and sequelae and what does it result in?

Answer 34

Inhalation of noxious stimuli (i.e smoke), caused by heat, particulates or irritants (i.e NH3) which leads to chemical tracheobronchitis, edema, pneumonia and ARDS

Question 35

How do many patients present with inhalation injury and sequelae? What might be observed on examination?

Answer 35

Secondary burns, CO inhalation, cyanide poisoning or arsenic poisoning. Singed nasal hairs or soot in the oropharynx are common on examination

Question 36

What might be noted on a bronchoscopy in inhalation injury and sequelae?

Answer 36

Severe edema, congestion of the bronchus and soot deposition

Question 37

What parts of the lungs do asbestos and silica affect?

Answer 37

Asbestos: base/lower lobes "Is from the roof but affects the base" Silica: Upper lobes "Is from the base (earth) but affects the roof"

Question 38

What increases your risk of getting asbestosis?

Answer 38

shipbuilding, roofing, plumbing

Question 39

What is pathognomonic of asbestosis?

Answer 39

"Ivory white" calcified supradiaphragmatic and pleural plaques

Question 40

What does asbestosis increase your risk of?

Answer 40

1. bronchogenic carcinoma (> mesothelioma) 2. Caplan syndrome (rheumatoid pneumoconiosis) 3. Pleural effusions

Question 41

How do asbestos particles appear histologically? Where and how can they be visualized and obtained?

Answer 41

Golden-brown fusiform rods, visualized in alveolar sputum using Prussian blue stain - and are often obtained via bronchoalveolar lavage

Question 42

What increases your risk of getting berylliosis? How does it appear histologically and how can it be treated?

Answer 42

Exposure to beryllium in aerospace and manufacturing industries. Noncaseating granulomas on histology and so is occasionally responsive to steroids

Question 43

Which part of the lung is affected by berylliosis and what can it increase your risk of?

Answer 43

The upper lobes, increased risk of cancer and cor pulmonale

Question 44

Describe the pathology of coal worker's pneumoconiosis, what can it increase your risk of?

Answer 44

Prolonged coal dust exposure -> leads to macrophages laden with carbon -> inflammation and fibrosis Increased risk of Caplan syndrome

Question 45

Which part of the lung is affected by coal worker's pneumoconiosis? How does it appear on imaging

Answer 45

Upper lobes, small rounded nodular opacities are seen on imaging

Question 46

What term is used to describe the asymptomatic nature of pneumoconiosis found in many urban dwellers exposed to sooty air

Answer 46

Arthracosis

Question 47

What increases your risk of getting silicosis? How does it appear on a CXR?

Answer 47

The Silly Egg sandwich I found is mine! Increased risk of getting it when: SANDblasting, FOUNDries, MINEs CXR: "EGGshell" calcifications of hilar lymph nodes

Question 48

How do macrophages respond to silica and what happens as a result of this?

Answer 48

They release fibrogenic factors leading to fibrosis

Question 49

What does silicosis increase your risk of?

Answer 49

Silica may also disrupt phagolysosomes and impair macrophages - increasing susceptibility to TB 3 Cs: Cancer, cor pulmonale, Caplan's syndrome