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Respiratory > EXAM #1: INTERSTITIAL LUNG DISEASE > Flashcards

EXAM #1: INTERSTITIAL LUNG DISEASE Flashcards

1
Q

What are interstitial lung diseases?

A

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”

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2
Q

What is the classic presentation of an interstitial lung disease?

A

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

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3
Q

How do the restrictive lung diseases present on PFT?

A
  • Decreased TLC
  • Decreased FEV1
  • Markedly decreased FVC

*Thus, FEV1/FVC ratio is INCREASED

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4
Q

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

A

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

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5
Q

What are the physical signs of a chronic lung disease?

A

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

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6
Q

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

A

N/A

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7
Q

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

A

Decreased DCLO

*Diffusing Capacity of the Lung for CO

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8
Q

What are the major classifications of the interstitial lung diseases?

A

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

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9
Q

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

A
  • Connective tissue
  • Drugs
  • Occupational
  • Hypersensitivity pneumonitis
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10
Q

What is the Granulomatous ILD?

A

Sarcoidosis

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11
Q

What is the major Idiopathic Interstitial Pneumonia?

A

Idiopathic Pulmonary Fibrosis (IPF)

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12
Q

What is Idiopathic Pulmonary Fibrosis?

A

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.

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13
Q

What is the prognosis for IPF?

A

Survival of 3-5 years from dx.

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14
Q

What are the clinical signs of IPF?

A

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

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15
Q

What are the CXR features of IPF?

A
  • Loss of intercostal spaces with inspiratory film
  • Reticular infiltrates
  • Fibrotic changes
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16
Q

How does IPF appear on HRCT?

A

“Honeycomb”

Corresponds with UIP/temporal heterogeneity

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17
Q

How is IPF medically managed (new)?

A

1) Pirfenidone
2) Nintedanib

Best response if given early in disease course

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18
Q

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

A

Ground-glass opacity

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

19
Q

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

A

Much better

20
Q

What is the medical treatment for NSIP?

A

Steroids

21
Q

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

A

Smoking

Note that RB-ILD present at a younger age

22
Q

How do DIP and RB-ILD present?

A

Young smokers, more common in males

23
Q

What is the treatment for DIP and RB-ILD?

A

Smoking cessation and steroids

24
Q

How do DIP and RB-ILD appear on CT?

A

Ground-glass opacity (may be diffuse or patchy)

V. similar to NSIP

25
Q

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

A

Rare fulminant diffuse lung injury seen in previously healthy individuals

*Leads to ARDS

26
Q

How does the CT of AIP/DAD appear?

A

Diffuse, bilateral air space and reticular infiltrates

*ARDS picture

27
Q

How is AIP/DAD managed?

A

Supportive care and potentially steroids

*Most develop respiratory failure requiring mechanical ventilation

28
Q

What is Cryptogenic Organizing Pneumonia (COP)?

A

Interstitial lung disease that resembles pneumonia but is unresponse to antibiotics

This is eventually diagnosed with alveolar wash and bronchoscopy

29
Q

What is the treatment for COP?

A

Corticosteroids

*Note that this has a GOOD prognosis

30
Q

How does COP appear on CT?

A

Patchy infiltrates with small nodular opacities

Subpleural and peribronchilar i.e. around the airways

31
Q

What is hypersensitivity pneumonitis?

A

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”

32
Q

Describe the pathogenesis of hypersensitivity pneumonitis.

A
  • Cell-mediated
  • CD8+ influx
  • Activation of alveolar macrophages
33
Q

How does hypersensitivity pneumonitis present acutely?

A

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

34
Q

How does subacute hypersensitivity pneumonitis compare to acute?

A

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

35
Q

How does chronic hypersensitivity pneumonitis compare to acute and subacute?

A

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

36
Q

How does chronic hypersensitivity pneumonitis appear on CT?

A

Ground glass opacification with centrilobular nodules

37
Q

What drugs are highly associated with drug-induced ILD?

A

1) Amiodarone
2) Nitrofurantoin
3) Bleomycin

38
Q

What is the treatment of amiodarone or nitrofurantoin induced ILD?

A

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.

39
Q

What is Acute Eosinophilic Pneumonia?

A

Pneumonia with eosinophils on bronchiolar lavage

*No eosinophils systemically

40
Q

What is Chronic Eosinophilic Pneumonia?

A

Pneumonia with EOSINOPHILIA and eosinophils present on bronchiolar lavage

41
Q

How does Chronic Eosinophilic Pneumonia appear on CT?

A
  • Subpleural consolidations and upper lobe involvement
  • “Negative” of pulmonary edema*

Pulmonary edema starts central and works out; this is the opposite

42
Q

What is lymphangioleiomyomatosis (LAM)?

A

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

43
Q

How does lymphangioleiomyomatosis appear on CT?

A

Diffusely distributed thin-walled cysts

44
Q

What are the diagnostic steps in evaluating ILD?

A

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

Respiratory (35 decks)

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