Obstructive and Restrictive Pulmonary Disease (Handorf)

Question 1

What is pulmonary emphysema?

Answer 1

irreversible enlargement of air spaces distal to the terminal bronchioles

Question 2

What are the two pathological causes of emphysema?

Answer 2

1. destruction of walls

2. dilation of alveoli

Question 3

Most significant etiology of emphysema

Answer 3

smoking

Question 4

Why does emphysema cause “more suffering” than lung cancer, according to Handorf?

Answer 4

more common
emphysema patients live longer
emphysema is progressive
some lung cancers can be cured

Question 5

Centrilobar pattern of emphysema is characteristic of:

Answer 5

smoking damage

Question 6

Panlobar pattern of emphysema is characteristic of:

Answer 6

alpha-1-antitrypsin deficiency

Question 7

Fundamental unit of lung; includes terminal br, resp br, alveolar duct and alveoli

Answer 7

acinus

Question 8

Centriacinar emphysema initially affects:

What usually causes this, and in what portion of the lungs?

Answer 8

respiratory bronchioles

smokers

upper lobes and apices

Question 9

Panacinar emphysema initially affects

What usually causes this, and in what portion of the lungs?

Answer 9

peripheral structures, such as alveoli and ducts; it later extends to affect resp bronchioles

alpha-1-antitrypsin deficiency
lower basal zones

Question 10

What is the pathophys of emphysema? How do the two causes overlap?

Answer 10

1. Smoking produces ROS, which signal neutrophils via IL8, LTB4, TNF
2. elastase (from neutrophils and resultant from inactivation of antiproteinases via ROS) damages tissue

smoking creates a function alpha-1-antitrypsin deficiency

Question 11

What is bullous emphysema?

How would you realize you had this?

Answer 11

damaged alveoli extend to form exceptionally large air spaces (especially in apices)

abnormal air space ruptures (often healthy young adults); fluid leaks into pleural space potentially causing lung collapse
*this happened to Zach, minus the pneumothorax.

Question 12

How is chronic bronchitis defined?

Answer 12

clinical condition characterized by persistent cough with sputum production for at least 3 months in at least 2 consecutive years

*Robbins defines it pathologically as mucus gland hypertrophy with hypersecretion and bronchiolitis

Question 13

What causes chronic bronchitis?

Answer 13

chronic irritation

microbiologic infections

Question 14

What is bronchiectasis?

Answer 14

permanent abn dilation of airways

Question 15

Where do chronic bronchitis and emphysema intersect?

Answer 15

both involve destruction of alveolar walls with inflammatory cell involvement

*is this right?

Question 16

Gross pathology of asthma

Answer 16

• pulm hyperexpansion, with and without atelectasis

- bronchial mucus plugs due to hypersecretion

Question 17

Micro pathology of asthma

Answer 17

–bronchial lumen mucus with: eosinophils, neutrophils, creola bodies, Curschmann spirals, Charcot-Leyden crystals

–airways remodeling: sm muscle hyperplasia and hypertrophy, submucosal gland expansion, thickened basement membrane, fibrosis

Question 18

What is pneumoconioses?

Answer 18

non-neoplastic lung reaction to inhalation of mineral dusts, org and inorg particulates, chemical fumes or vapors

Question 19

Types of pneumoconioses

Answer 19

1. coal miners lung (anthracosis to prog massive fibrosis; large blackened scars)
2. silicosis (most common)
3. asbestosis
4. berylliosis
5. farmers lung (bagassosis, org dust)
6. byssinosis (org dust exposure + asthma)

Question 20

Causes fibrocalcific plaques on the pleural side of the diaphragm

Answer 20

pneumoconioses from asbestos

Question 21

What causes ferruginous bodies?

Answer 21

inhaled asbestos particles which are ingested by macrophages and coated with iron salts

Question 22

What is sarcoid characterized by?

Answer 22

NONcaseating granulomas in many tissues and organs

Question 23

Who is the STEP patient with sarcoidosis?

Answer 23

middle aged AAF

Question 24

Where are sarcoid lesions commonly found?

Answer 24

lungs
eyes
skin
lymph nodes

Question 25

What is likely the cause of sarcoidosis?

Answer 25

infectious, organic or inorganic particles begin an immune reaction

Question 26

What are strong contributors to sarcoidosis susceptibility?

Answer 26

macrophage HLA and BTNL2 alleles

Question 27

What initiates the formation and maintenance of sarcoid granulomas?

Answer 27

CD4 T cells

Question 28

What causes progressive fibrosis in certain sarcoidosis patients?

Answer 28

Th2 activated alveolar macrophages which stimulate fibroblast prolif and collagen production

Question 29

What are characteristics of advanced sarcoidosis?

Answer 29

bronchiectasis, emphysema, fibrosis, pulm HTN