17 - Interstitial Lung Disease Pathology

Question 1

What is the gross and histological appearance of fibrosis?

Answer 1

Gross: firm, white/tan

Histology: distorted architecture, alveolar septae are thickened with collagen

Question 2

Asbestosis affects predominantly [upper/lower] lobes.

Answer 2

lower

Question 3

Silicosis affects predominantly [upper/lower] lobes.

Answer 3

upper

Question 4

Coal worker pneumoconiosis affects predominantly [upper/lower] lobes.

Answer 4

upper

Question 5

What occupations are at high risk for silicosis?

Answer 5

• quartz workers
• foundry workers
• sandblasters
• stone cutters

Question 6

Small particles seen on polarized light is suggestive of ___.

Answer 6

silicosis

Question 7

Fibrotic nodules that coalesce, resembling a granuloma but with collagen in the center suggest ___.

Answer 7

silicosis

Question 8

If someone has silicosis, what do they have an increased risk for?

Answer 8

tuberculosis

Question 9

Lymphocytic infiltrate around bronchioles plus poorly formed, non-necrotizing granulomas (with giant cells and epitheloid histiocytes) are suggestive of ___.

Answer 9

hypersensitivity pneumonitis

Question 10

Hilar adenopathy and well-formed, non-necrotizing granulomas (with giant cells) are suggestive of ___.

Answer 10

sarcoidosis

Question 11

What is granulomatosis with polyangiitis?

Answer 11

vasculitis

Question 12

Granulomatous vasculitis with histiocytes and necrosis with nuclear debris from degenerating neutrophils suggest ___.

Answer 12

granulomatosis with polyangiits

Question 13

Which respiratory condition is C-ANCA positive?

Answer 13

granulomatosis with polyangiitis

Question 14

How do you treat granulomatosis with polyangiitis?

Answer 14

immunosuppressive therapy including steroids

Question 15

Stellate lesions, histiocytes with longitudinal grooves, eosinophils, and Birbeck granules suggest ___.

Answer 15

pulmonary Langerhans cell histiocytosis

Question 16

What immune cell is positive for the S-100 stain? What respiratory illness is this associated with?

Answer 16

Langerhans cells –> Langerhans cell histiocytosis

Question 17

What is the most common interstitial pulmonary fibrosis?

Answer 17

usual interstitial pneumonia (UIP)

Question 18

What are the two categories of causes of UIP?

Answer 18

• end process of many ILDs (asbestosis, chronic hypersensitivity pneumonitis, sarcoidosis, collagen vascular disease, etc.)
• idiopathic –> idiopathic pulmonary fibrosis

Question 19

Spatial and temporal heterogeneity and honeycombing is suggestive of ___.

Answer 19

usual interstitial pneumonia

Question 20

What is temporal heterogeneity?

Answer 20

Old, pink, dense collagen lesions + young, light lesions with fibroblastic foci and spindle cells

Question 21

What is spatial heterogeneity?

Answer 21

Some lung areas are diseased, others look normal

Question 22

Plugs of fibroblastic tissue within bronchioles and extended to alveoli suggest ___.

Answer 22

cryptogenic organizing pneumonia (COP)

Question 23

What are the causes of cryptogenic organizing pneumonia?

Answer 23

• idiopathic
• medications
• can follow some pneumonias
• rheumatologic diseases

Question 24

How do you treat cryptogenic organizing pneumonia?

Answer 24

steroids

Question 25

Diffuse alveolar damage with hyaline membranes is suggestive of ___.

Answer 25

acute interstitial pneumonia (and ARDS)

Question 26

What does AIP stand for?

Answer 26

acute interstitial pneumonia

Question 27

Acute interstitial pneumonia has a [low/high] mortality.

Answer 27

high

Question 28

Alveoli filled with alveolar macrophages with brown pigment and minimal fibrosis are suggestive of ___.

Answer 28

DIP

Question 29

What is the treatment for DIP?

Answer 29

• stop smoking

- steroids (if smoking cessation is not effective)