Interstitial Lung Disease Flashcards

1
Q

What is the interstitium?

A

The lung interstitium is the space between the air sacs and the small blood vessels that surround the air sacs. It contains connective tissue. When you breathe, oxygen from the air passes through your air sacs and lung interstitium and into your blood.

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2
Q

What is the most common type of interstitial lung disease?

A

Idiopathic

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3
Q

What is the epidemiology of interstitial lung disease?

A
  • Not well defined, diagnosis of exclusion
  • Males slightly higher than females
  • Increased risk with +FH
  • Associated with occupational exposures and autoimmune diseases
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4
Q

What is the presentation of interstitial lung disease?

A
  • Progressive exertional dyspnea (m/c)
  • Dry cough
  • May have Sx of autoimmune disease
  • Can be asymptomatic

Less common Sx include pleuritic chest pain or hemoptysis

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5
Q

What are the physical exam findings of interstitial lung disease?

A

Non-specific PE findings include:
- Bibasilar crackles
- Clubbing
- PE consistent with pulmonary hypertension
- Tachypnea
- Reduced chest pain

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6
Q

What is the most important element of the work-up for interstitial lung disease?

A

Thorough history
- Occupation
- Family history
- PMH (connective tissue diseases, malignancy, rheum conditions)

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7
Q

What is the usual first line imaging in working-up interstitial lung disease?

A

CXR (supportive but not diagnostic)

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8
Q

What is the best imaging test for ILD?

A

High-resolution CT

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9
Q

What will PFT’s show in ILD?

A

Maybe normal early on, later ILD will show a restrictive pattern

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10
Q

What are some supportive CXR and CT findings of ILD?

A

Bronchiectisis (tram-track appearance)
Miliary pattern
Honeycombing
Kerley lines
Reticular or rediculonodular opacities

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11
Q

What is the goal of treatment for ILD?

A

Prevent/limit progression of fibrosis. Fibrosis is irreversible.

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12
Q

What are the pharmacological treatments for ILD?

A
  • Prednisone
  • Antifibrotic agents (idiopathic pulmonary fibrosis)
  • Treat GERD
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13
Q

What is the only definitive treatment for refractory disease?

A

Lung transplant

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14
Q

What is the characteristic “usual interstitial pneumonia” pattern on imaging in idiopathic pulmonary fibrosis?

A
  • Basilar reticular opacities
  • Honeycombing
  • Bronchiectasis (tram tracks)
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15
Q

What is the epidemiology of sarcoidosis?

A
  • Systemic inflammatory disorder
  • Incidence higher in black patients
  • F > M
  • Average age of dx 20-40s (second peak around 60)
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16
Q

What is the characteristic finding on imaging in sarcoidosis?

A

Non-caseating granulomas

17
Q

Does TB have caseating or non-caseating granulomas?

A

Caseating granulomas

18
Q

What is needed for definitive diagnosis of sarcoidosis?

A

Biopsy

19
Q

What is pathognomonic for sarcoidosis?

A

Non-caseating granulomas

20
Q

Idiopathic pulmonary fibrosis ultimately destroys what cell type?

A

Type I endothelial cells and overgrowth of type II (this does not allow for repair/regrowth of the damaged epithelium)

21
Q

What is melanoptysis?

A

black hemoptysis (mucus)