SM 167a Lung Pathology & Restrictive Lung Disease

Question 1

What 2 areas should be examined when looking at a Lung?

Answer 1

Surface of the Lung + Cut Surface

Question 2

How should the surface of the Lung appear?

Answer 2

Smooth + Glistening + No Adhered Structures

Question 3

How should the cut surface of the Lung appear?

Answer 3

Color = Red/Maroon + Consistency = Sponge-like + Air Spaces = Patent/Wide Open/Few/Central

Question 4

What colors of the Lung are worrisome?

Answer 4

Red Lung cut surface = hemorrhage Grey Lung cut surface = Bad

Question 5

What type of cell is found on the epithelium of larger Airway histology?

Answer 5

Cilliated Columnar Epithelium

Question 6

What differentiated Bronchi and Bronchioles?

Answer 6

Bronchi have Cartilage Rings + Submucosal glands while Bronchioles do not

Question 7

What makes up an Alveolar Septae?

Answer 7

Type I Pneumocyte + Capillary + Type I Pneumocyte = Septae (Very Thin)

Question 8

What are the two categories of Chronic Lung Disease?

Answer 8

Obstructive Lung Disease + Restrictive Lung Disease

Question 9

What are the general properties of Obstructive Lung Disease?

Answer 9

Involve the airway, increases resistance to airflow, and does not progress to “honeycomb lung”

Question 10

What are the general properties of Restrictive Lung Disease?

Answer 10

Involve the lung parenchyma, decreased expansion of lung parenchyma via fibrosis leading to decreased lung capacity, and progresses to “honeycomb lung”

Question 11

What pathophysiology is associated with Restrictive Lung Disease?

Answer 11

Restrictive Lung Disease often involves Fibrosis, which prevents Alveoli from expanding

Question 12

What is “honeycomb lung”?

Answer 12

End stage restrictive lung disease

Question 13

What is categorized as Obstructive Lung Disease?

Answer 13

CAB = COPD (Emphysema and Chronic Bronchitis), Asthma, Bronchiectasis

Question 14

What is categorized as Restrictive Lung Disease?

Answer 14

HUNS = Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, Nonspecific Interstitial Pneumonia, and Sarcoidosis

Question 15

Which forms of Restrictive Lung Disease are patterns?

Answer 15

Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP), they are not diseases but a pattern of injury that result from many other diseases

Question 16

Which forms of Restrictive Lung Disease are specific disease entities?

Answer 16

Sarcoidosis and Hypersensitivity Pneumononitis

Question 17

Is UIP a disease or a pattern?

Answer 17

UIP is a pattern

Question 18

What are common causes of UIP?

Answer 18

IPF, Connective Tissue diseases (Rheumatoid Arthritis), Asbestosis

Question 19

What is the worst cause of UIP?

Answer 19

IPF - progressive disease with 3 year survival

Question 20

What is the pathology of UIP?

Answer 20

“Fibroblastic Foci” with temporal and geographic heterogeneity

Question 21

Which disease presents with temporal and geographic heterogeneity and what does that mean?

Answer 21

UIP; in UIP, areas of young and old fibrosis are next to eachother as well as areas of fibrotic and healthy lung tissue in general

Question 22

Where does UIP tend to target?

Answer 22

Subpleural regions

Question 23

Is NSIP a specific disease or a pattern?

Answer 23

NSIP is a pattern

Question 24

What are the common causes of NSIP?

Answer 24

Autoimmune diseases like Rheumatoid Arthritis and Sarcoidosis and Idiopathic NSIP

Question 25

What is the pathology of NSIP?

Answer 25

Temporally and geographically uniform fibrosis

Question 26

How do the pathologies of NSIP and UIP compare?

Answer 26

UIP is heterogenous both temporally and geographically while NSIP is uniform

Question 27

What is Sarcoidosis?

Answer 27

A granulomatous disease of unknown etiology

Question 28

Who is typically affected by Sarcoidosis?

Answer 28

Adults < 40 y/o, Females, and Black Patients

Question 29

Which areas does Sarcoidosis effect?

Answer 29

Sarcoidosis effects the Lungs as well as other systemic areas like the eyes and skin

Question 30

How does Sarcoidosis appear on pathology?

Answer 30

Well formed granulomas without lymphoid inflammation or necrosis

Question 31

Where does Sarcoidosis effect the lungs?

Answer 31

Sarcoidosis typically effects the airways and veins

Question 32

What must be confirmed before Sarcoidosis is diagnosed?

Answer 32

Infectious etiologies must be ruled out first

Question 33

What cells are in a Granuloma?

Answer 33

Balls of Histocytes if sterile, add immune cells if infected

Question 34

What is Hypersensitivity Pneumonia?

Answer 34

A pneumonia caused by prolonged exposure to organic antigens

Question 35

How does Hypersensitivity Pneumonia present?

Answer 35

Acute = fever/cough/dyspnea + chronic = respiratory failure

Question 36

Is Hypersensitivity Pneumonia chronic or acute?

Answer 36

Both

Question 37

How does Hypersensitivity Pneumonia appear on pathology?

Answer 37

Poorly formed non-necrotizing granulomas

Question 38

What type of pattern of injury does chronic Hypersensitiivty Pneumonia display?

Answer 38

UIP

Question 39

What is Honeycomb Lung?

Answer 39

End stage of fibrosis associated restrictive lung disease, especially UIP

Question 40

How is Honeycomb Lung diagnosed?

Answer 40

CT Scans and specimens diagnose Honeycomb Lung

Question 41

What does Honeycomb Lung look like?

Answer 41

Cystic spaces lined with columnar bronchial epithelial cells; dense fibrosis

Question 42

How can Honeycomb Lung be differentiated from Emphysema?

Answer 42

Emphysema lacks fibrosis

Question 43

When can IPF be diagnosed?

Answer 43

IPF is diagnosed if the UIP patterns of Temporal and Geographic heterogeneity in fibrosis is present and no clear cause can be found

Question 44

Is sarcoidosis systemic or organ-based?

Answer 44

Sarcoidosis is a systemic disease that affects multiple organs

Question 45

What is needed before sarcoidosis can be diagnosed?

Answer 45

Special stains and cultures