EXAM #1: INTERSTITIAL LUNG DISEASE

Question 1

What are interstitial lung diseases?

Answer 1

A group of disorders characterized by cellular infiltration, scarring, or structural disruption of the lung parenchyma

*These are also known are the “restrictive lung diseases”

Question 2

What is the classic presentation of an interstitial lung disease?

Answer 2

1) Progressive dyspnea on exertion
2) Dry cough
3) End-inspiratory crackles

Question 3

How do the restrictive lung diseases present on PFT?

Answer 3

• Decreased TLC
• Decreased FEV1
• Markedly decreased FVC

*Thus, FEV1/FVC ratio is INCREASED

Question 4

What are the historical features that should make you suspicious for a restrictive lung disease?

Answer 4

1) Occupational exposure
2) Drugs e.g. “Bleomycin, Amiodarone, and Nitrofunatin”
3) Connective tissue disorder
4) Cigarette smoking
5) Family history of similar disease

Question 5

What are the physical signs of a chronic lung disease?

Answer 5

Signs of long-term hypoxia

1) Clubbing
2) Tachypnea*
3) Dry crackles
4) Increased right-heart pressure
- P2
- JVD
- Edema
5) Skin and soft tissue changes

*Small TLC= increased rate

Question 6

Draw the volume-time graph that is characteristic of the restrictive lung diseases.

Answer 6

N/A

Question 7

What is one of the earliest signs of restrictive lung disease on PFT?

Answer 7

Decreased DCLO

*Diffusing Capacity of the Lung for CO

Question 8

What are the major classifications of the interstitial lung diseases?

Answer 8

1) Known association
2) Granulomatous
3) Idiopathic Interstitial Pneumonias
4) Misc.

Question 9

What are the four types of ILD with a known association?

Answer 9

• Connective tissue
• Drugs
• Occupational
• Hypersensitivity pneumonitis

Question 10

What is the Granulomatous ILD?

Answer 10

Sarcoidosis

Question 11

What is the major Idiopathic Interstitial Pneumonia?

Answer 11

Idiopathic Pulmonary Fibrosis (IPF)

Question 12

What is Idiopathic Pulmonary Fibrosis?

Answer 12

Fibrosis of the lung interstitium*

*A collection of support tissues within the lung that includes the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues.

Question 13

What is the prognosis for IPF?

Answer 13

Survival of 3-5 years from dx.

Question 14

What are the clinical signs of IPF?

Answer 14

1) Exertional dyspnea
2) Non-productive cough
3) Inspiratory “velcro-like” crackles
4) Clubbing

Question 15

What are the CXR features of IPF?

Answer 15

• Loss of intercostal spaces with inspiratory film
• Reticular infiltrates
• Fibrotic changes

Question 16

How does IPF appear on HRCT?

Answer 16

“Honeycomb”

Corresponds with UIP/temporal heterogeneity

Question 17

How is IPF medically managed (new)?

Answer 17

1) Pirfenidone
2) Nintedanib

Best response if given early in disease course

Question 18

How does Non-specific Interstitial Pneumonitis (NSIP) appear on CT?

Answer 18

Ground-glass opacity

*Note that vs. IPF this is “homogenous vs. heterogenous” and honeycombing is uncommon

Question 19

How does the prognosis of Non-specific Interstitial Pneumonia (NSIP) compare to IPF?

Answer 19

Much better

Question 20

What is the medical treatment for NSIP?

Answer 20

Steroids

Question 21

What are Desquamative Interstitial Pneumonitis (DIP) and Respiratory Bronchiolitis-associated Interstitial Lung Disease (RB-ILD) associated with?

Answer 21

Smoking

Note that RB-ILD present at a younger age

Question 22

How do DIP and RB-ILD present?

Answer 22

Young smokers, more common in males

Question 23

What is the treatment for DIP and RB-ILD?

Answer 23

Smoking cessation and steroids

Question 24

How do DIP and RB-ILD appear on CT?

Answer 24

Ground-glass opacity (may be diffuse or patchy)

V. similar to NSIP

Question 25

What is Acute Interstitial Pneumonitis/ Diffuse Alveolar Damage (AIP/DAD)?

Answer 25

Rare fulminant diffuse lung injury seen in previously healthy individuals *Leads to ARDS

Question 26

How does the CT of AIP/DAD appear?

Answer 26

Diffuse, bilateral air space and reticular infiltrates *ARDS picture

Question 27

How is AIP/DAD managed?

Answer 27

Supportive care and potentially steroids *Most develop respiratory failure requiring mechanical ventilation

Question 28

What is Cryptogenic Organizing Pneumonia (COP)?

Answer 28

Interstitial lung disease that resembles pneumonia but is unresponse to antibiotics *This is eventually diagnosed with alveolar wash and bronchoscopy*

Question 29

What is the treatment for COP?

Answer 29

Corticosteroids *Note that this has a GOOD prognosis

Question 30

How does COP appear on CT?

Answer 30

Patchy infiltrates with small nodular opacities *Subpleural and peribronchilar i.e. around the airways*

Question 31

What is hypersensitivity pneumonitis?

Answer 31

A granulomatous reaction to inhaled organic antigens, esp. 1) Pigeon droppings 2) Farmers with actinomyces in grain silos *Note that this is also called "Farmer's Lung"

Question 32

Describe the pathogenesis of hypersensitivity pneumonitis.

Answer 32

- Cell-mediated - CD8+ influx - Activation of alveolar macrophages

Question 33

How does hypersensitivity pneumonitis present acutely?

Answer 33

Fever, cough, and dyspnea after exposure--remission after removal of antigen

Question 34

How does subacute hypersensitivity pneumonitis compare to acute?

Answer 34

Gradual onset over weeks of exposure *Note that this subset of the patient population will have radiographic evidence of disease, and may require require glucocorticoids in addition to removal from antigen*

Question 35

How does chronic hypersensitivity pneumonitis compare to acute and subacute?

Answer 35

1) Long-term onset without acute episodes 2) May have clubbing 3) Fibrosis

Question 36

How does chronic hypersensitivity pneumonitis appear on CT?

Answer 36

Ground glass opacification with centrilobular nodules

Question 37

What drugs are highly associated with drug-induced ILD?

Answer 37

1) Amiodarone 2) Nitrofurantoin 3) Bleomycin

Question 38

What is the treatment of amiodarone or nitrofurantoin induced ILD?

Answer 38

1) D/c amiodarone 2) Steroids *Note the half-life of amiodarone--takes 2 months to clear; thus, if you only d/c med they will likely continue to have sx.*

Question 39

What is Acute Eosinophilic Pneumonia?

Answer 39

Pneumonia with eosinophils on bronchiolar lavage *No eosinophils systemically

Question 40

What is Chronic Eosinophilic Pneumonia?

Answer 40

Pneumonia with EOSINOPHILIA and eosinophils present on bronchiolar lavage

Question 41

How does Chronic Eosinophilic Pneumonia appear on CT?

Answer 41

- Subpleural consolidations and upper lobe involvement - "Negative" of pulmonary edema* *Pulmonary edema starts central and works out; this is the opposite*

Question 42

What is lymphangioleiomyomatosis (LAM)?

Answer 42

Progressive cystic lung disease that exclusively affects women of child-bearing age *Note that this is very severe and typically, the only treatment is lung transplant

Question 43

How does lymphangioleiomyomatosis appear on CT?

Answer 43

Diffusely distributed thin-walled cysts

Question 44

What are the diagnostic steps in evaluating ILD?

Answer 44

1) CXR, labs PFT 2) HRCT 3) Bronchoscopy 4) Biopsy