Restrictive/Inflammatory Disease

Question 1

What are the hallmarks of restrictive disease?

Answer 1

Reduced compliance and elastic recoil pressure needed. Compliance can be altered in the lungs or the chest wall.

Question 2

What 3 mechanisms reduce lung compliance?

Answer 2

1) Increased thickness of lung interstitium (fibrosis or inflammation)
2) Increased lung water (edema)
3) Increased alveolar surface tension (water, surfactant deficiency, injury/ARDS)

Question 3

Give an example of decreased chest wall compliance at each layer

Answer 3

Skin- Burns
Soft Tissue- Obesity
Bone- Scoliosis
Muscle- Neuromuscular disorders
Ligament- Ankylosing spondylitis
Pleural cavity- Pleural effusion
Pleura- Fibrosis/thickening

Question 4

What differences in lung and chest wall compliance can be seen in PFTs?

Answer 4

Both lung and chest wall problems cause restrictive PFT patterns (normal ratio, low volumes).
Lung issues cause a low corrected DLCO
Chest wall issues cause a normal corrected DLCO

Question 5

How does mixed obstructive-restrictive disease present in PFTs?

Answer 5

Low ratio, FRC, TLC and DLCO

Question 6

What is the presentation of Idiopathic Pulmonary Fibrosis?

Answer 6

Insidious onset DOE, non-productive cough.
Caused by disordered alveolar repair.
Doesn’t respond to anti-inflammatory agents

Question 7

What are the pathologic differences between acute, eosinophilic, and organizing pneumonia?

Answer 7

Acute: neutrophils
Eosinopliic: eosinophils
Organizing: loose myxoid fibroblastic tissue

Question 8

What are the four main smoking pathologies?

Answer 8

1) Centribular emphysema (upper lobes)
2) Respiratory bronchiolitis
3) Desquamative interstitial pneumonia
4) Pulmonary Langerhan’s Cell Histiocytosis

Question 9

Describe Usual Interstitial Pneumonia (UIP)

Answer 9

Patchy heterogeneous fibrosis, fibroblastic foci, honeycombing

Question 10

Describe Non-Specific Interstitial Pneumonia (NSIP)

Answer 10

Homogenous, no honeycombing or foci

Question 11

Describe Hypersensitivity Pneumonia

Answer 11

Airway inflammation with non-necrotizing granulomas is response to an antigen/allergen.

Question 12

What’s the appearance of Small Cell Carcinoma?

Answer 12

Small blue “crush” figures, lots of DNA, necrosis. Centrally located. SMOKING.

Question 13

What’s the appearance of Squamous Cell Carcinoma?

Answer 13

Keratin pearls, cavitation, centrally located. Smoking.

Question 14

What is the appearance of Adenocarcinoma?

Answer 14

Gland-like structures, peripheral tumors.