COPD and Restrictive Lung Disease

Question 0

What can cause both inspiratory and expiratory obstructive lung disease?

Answer 0

Fixed intra or extra thoracic obstruction

Question 1

What can cause inspiratory obstructive lung disease?

Answer 1

Snoring

Foreign body

Question 2

What can cause an expiratory obstructive lung disease?

Answer 2

Asthma
COPD 
Bronchiectasis
Bronchiolitis
Broncheomalacia

Question 3

What is the mechanism of obstruction in asthma?

Answer 3

Bronchospasm
Inflammation
Mucus

Question 4

What is the mechanism of obstruction in chronic bronchitis?

Answer 4

Bronchospasm
Inflammation
Mucus

Question 5

What is the mechanism of obstruction in emphysema?

Answer 5

Loss of lung elastic recoil

Question 6

What are the main symptoms of obstructive lung disease?

Answer 6

Dyspnea
Cough - in asthma and chronic bronchitis, not emphysema
Wheezing

Question 7

What are some important questions to ask a patient with suspected obstructive lung disease?

Answer 7

Dyspnea - when did it start and get worse
Cough - productive?
Smoking history
History of allergy
History of wheezing - not in pure emphysema

Question 8

What are physical findings to focus on when evaluating patients with obstructive lung disease?

Answer 8

RR
Cyanosis - 5 grams unsaturated hemoglobin/100 cc of blood, more hemoglobin shows this more readily, rarely in asthma or emphysema
Decreased breath sounds (asthma indicate attack, common in emphysema), wheezing or rhonchi (rare inspiratory in stable asthma or COPD, expiratory more common, no rhonchi in emphysema)
Rales should not be heard - dry suggest restriction like fibrosis

Question 9

What is the alveolar air equation?

Answer 9

PAO2 = 150 - (PaCO2/.8)

Question 10

What is the hallmark of obstructive lung disease?

Answer 10

FEV1/FVC less than 70% AND FEV1 less than 80%

Question 11

What is the Ddx of a fixed obstruction?

Answer 11

Laryngeal carcinoma
Thyromegaly
Vocal cord asthma

Question 12

What is the Ddx of a variable extra thoracic obstruction?

Answer 12

Obstructive sleep Alena
Tracheomalacia
Inhibits inspiratory limb of flow volume loop

Question 13

What is the Ddx of a variable intrathoracic obstruction?

Answer 13

Asthma
COPD
Bronchiectasis
Affects expiratory limb on flow volume loop

Question 14

What DLCO means patient will develop arterial hypoxemia with exercise?

Answer 14

<45%

Question 15

When is an ABG ordered?

Answer 15

When FEV1 less than or equal to 30-40% predicted

Normal pCO2 of 41 is bad sign in someone with asthma - developing respiratory acidosis (should be lower)

Question 16

What ABGs are present in COPD?

Answer 16

Emphysema usually has pCO2 < 45 and moderate hypoxemia (60-70) on room air at rest
Chronic bronchitis have pCO2 > 45 and pH only marginally low due to compensatory metabolic alkalosis, pO2 often <7.31) indicates acute worsening of COPD with worsening CO2 retention

Question 17

What are interstitial lung diseases?

Answer 17

Affect both alveolar and interstitial compartments

Lead to restriction

Question 18

What are the five categories of ILDs?

Answer 18

Idiopathic interstitial pneumonias
Connective tissue diseases
Drug induced diseases
Other systemic disorders
Occupational and environmental exposures
Last two are granulomatous

Question 19

What are signs and symptoms of ILDs?

Answer 19

Dyspnea
Tachypnea more prominent with exercise
Normal PaCO2, decreased PaO2, increased A-a gradient
Exercise induced hypoxemia sensitive for early disease

Question 20

Crackles, clubbing, cyanosis

Answer 20

Interstitial lung disease

Question 21

Wheezes, prolonged expiratory phase, distant breath sounds

Answer 21

Obstructive lung disease, including asthma

Question 22

Tachycardia, chest pain, leg swelling, syncope

Answer 22

Pulmonary emboli

Question 23

S3 gallop, bibasilar inspiratory crackles, PND, orthopnea

Answer 23

Cardiac disease

Question 24

Pale conjunctiva, tachycardia

Answer 24

Anemia

Question 25

What are HRCT findings and what do they indicate?

Answer 25

Ground glass appearance - alveolitis which can be reversible | Dense lines and honey combing - fibrosis, irreversible

Question 26

What are common causes of drug induced ILD?

Answer 26

*Chemo - bleomycin, busulfan, cyclophosphamide, methotrexate, nitrosoureas Cardiac - procainemide, amiodarone*, hydralazine Antibiotics - isoniazid, nitrofurantoin* Illicit drugs - heroine, cocaine

Question 27

Where are crackles less common?

Answer 27

Sarcoidosis

Question 28

Where is clubbing especially predominant?

Answer 28

IPF | Asbestosis

Question 29

What is loffgren's syndrome?

Answer 29

``` Erythema nodosum (painful nodular panniculitis and vasculitis), bilateral hilar adenopathy and arthralgias Associated with sarcoidosis ```

Question 30

What is Heerfordts syndrome?

Answer 30

Facial nerve palsy, parotidis, uveitis | Associated with sarcoidosis

Question 31

What is the natural history of sarcoidosis?

Answer 31

Therapy withheld until evidence of vital organ dysfunction - then corticosteroids, with or without immunosuppressants 1/3 spontaneously remit in 3 yrs, 1/3 progress over 5-10 yrs, 1/3 remain stable

Question 32

What mimics sarcoidosis?

Answer 32

Berylliosis - a pneumoconiosis

Question 33

How is hypersensitivity pneumonitis a mimic?

Answer 33

Acute resemble pneumonia Subacute resemble tb Chronic resemble IPF

Question 34

What are the shared clinical features of pneumoconioses?

Answer 34

Inhalation of inorganic material Linear dose response Long latency Symptoms and disease limited to respiratory system No known treatment except removal from exposure Very slow progression

Question 35

What are connective tissue diseases associated with ILD?

Answer 35

``` SLE RA Systemic sclerosis / CREST syndrome Sjogrens Dermatomyositis / poly myosotis ```

Question 36

What are hallmarks of connective tissue diseases and ILD?

Answer 36

Underlying disease usually present before lung involvement Multisystem involvement Younger age of onset

Question 37

What is idiopathic pulmonary fibrosis?

Answer 37

Chronic progressive dyspnea and cough, restrictive lung physiology, and UIP histologic pattern Characterized not by inflammation, but by chronic fibroproliferation due to abnormal lung tissue wound healing Rare in children, typically over 50, common in men and smokers Exclude other ILD causes Peripheral distribution Reticulations in lower lobes, honeycombing and traction bronchiectasis

Question 38

Why is it important to distinguish IPF from other ILDs?

Answer 38

IPF is the most lethal and doesn't respond to corticosteroids and immunosuppressants Only possible treatment is transplant but this is rare

Question 39

What is the molecular basis for the familial form of IPF?

Answer 39

AS pattern with incomplete penetrate Same presentation as clinical IPF but earlier age of onset Heterozygous mutations in genes encoding telomerase Mutations in gene encoding surfactant proteins A2 and C