11.20: Pulm III Flashcards Preview

MHD VI: Pulm > 11.20: Pulm III > Flashcards

Flashcards in 11.20: Pulm III Deck (51)
1

What is decreased TLC consistent with?

Restrictive lung disease

2

2 categories of restrictive lung disease?

1. Chest wall disorder
2. Chronic interstitial and infiltrative disease

3

Characteristics of interstitial lung diseases?

- Dyspnea
- End inspiratory crackles
- Hypoxia
- Nodules and irregular lines on cxr

4

2 Main categories and 3 main types of chronic interstitial lung disease?

1. Fibrosing
a. Usual interstitial pneumonia: 15%
b. Pneumoconiosis: 25%
2. Granulomatous
a. Sarcoidosis: 20%

5

What is interstitial lung disease?

- Inflammatory and fibrotic disease of alveolar walls and septa leading to reduced pulmonary compliance

6

What will interstitial lung disease lead to?

- Pulmonary htn.
- Cor pulmonale
"Honey comb lung" is end stage pathology

7

What is honeycomb lung characteristic of?

End stage restrictive interstitial lung disease

8

Difference between interstitial lung disease and emphysema?

There is fibrosis seen in interstitial disease but not in emphysema

9

What is idiopathic pulmonary fibrosis?

"IPF"
- Clinical term for "usual interstitial pneumonia"

10

Pathogenesis of IPF?

- Repeated epithelial injury leading to TGFbeta
- TGFB leads to fibroblasts
- Fibroblasts deposit collagen and ECM
- Pulmonary fibrosis ensues

11

Another name for idiopathic pulmonary fibrosis?

"IPF"
- Usual interstitial Pneumonia "UIP"

12

Another name for Usual interstitial Pneumonia "UIP"?

"IPF"
- Idiopathic pulmonary fibrosis

13

Characteristics of fibrosis in IPF?

- Non uniform pattern
- Varies in intensity and age
- Early "exuberant" fibroblastic proliferation

14

What is patchy interstitial fibrosis indicative of?

IPF

15

What is the impact of fibrosis in IFP?

- Patchy fibrosis destroys interstitial architecture
- Contraction of scarring pulls to destroy alveolar spaces
- Cystic spaces are created with Type II pneumocytes
"Honeycomb fibrosis"

16

What is cobblestone lung indicative of?

- IPF

17

Characteristics of IPF?

- Variable fibrosis more common in lower lobes
- "Cobblestone lung"
- Fibrosis more common in subpleural layers than in paraseptal

18

Clinical appearance of IPF?

- 40 - 70
- M > W
- Dyspnea on exertion
- Dry cough
- Velcro crackles

19

Prognosis of IPF/UIP? Treatment?

- Rapid deterioration with mean survival

20

What is pneumoconioses?

Accumulation of particulate, chemical fume, or vapor in lung and the reaction to its presence

21

3 main types of pneumoconioses?

1. Anthracosis: coal dust
2. Silicosis: silica from sand blasting
3. Asbestosis: ships, roofing, plumbing

22

What causes largest risk for development of pneumoconioses?

- Small 1 to 5 micrometer particles have greats danger of settle in linings of small airways and sacs and fester
- Too small to be caught by cilia and mucus
- Too big to be engulfed by macs

23

How is most inhaled particulate removed?

- Trapped in mucus and removed by ciliary elevator if > 1 microns

24

Pathogenesis of pneumoconiosis?

- Macs endocytose particles releasing inflammatory mediators
- Fibrogenesis and collagen deposition ensues

25

Presentation of anthracosis?

- Seen in coal, urban, and smokers
- Inhaled carbon pigment engulfed by interstitial macs
- Accumulates in tissues them moves to lymph
- Will see some black on autopsy but not other problems
** NO SYMPTOMS NOTES

26

Pathogenesis of simple coal workers pneumoconiosis?

- Same carbon irritant forms macules / nodules
- Patchy fibrosis noted but not extensive

27

Pathogenesis of progressive massive fibrosis?

"Black lung"
- Coalescence of coal nodules into large scars
- Fibrotic, bulky, heavily pigmented black tissue masses

28

Clinical course of coal workers pneumoconiosis?

Bening with progression to pulmonary dysfunction, pulm htn, or cor pulmonale

29

What is Caplan syndrome?

"Rheumatoid pneumoconiosis"
- Ptn. w/ rheumatoid arthritis exposed to coal
- Get fibrotic lesions with cavitating nodules on radiology
- Hints that RA alters bodies response to coal

30

Who is likely to get silicosis?

- Most prevalent occupational disease in world
- Workers in foundries, sandblasting, mines
- Caused by inhalation of crystalline silica engulfed by mac releasing fibrotic mediators

31

Appearance of silicosis?

- Nodules of "Whorled" hyalinized collagen
- Nodules can coalesce leading to massive fibrosis extending to hilar lymph nodes and pleura
"Eggshell calcification" of lymph nodes

32

When is egg shell calcification of lymph nodes seen?

- Silicosis

33

What does silicosis lead to increased susceptibility to?

- Tuberculosis
- Silicosis suppresses cell mediated immunity and impairs macrophage phagocytosis

34

What can silicosis lead to?

1. Dyspnea
2. Pulm htn
3. Cor pulmonale

35

Disease often associated with silicosis?

TB

36

Disease often associated with coal workers?

Rheumatoid arthritis

37

Which form of Pneumoconioses is carcinogenic?

Asbestosis

38

Why is asbestos often used?

1. Cheap
2. Durable
3. Head resistant for insulation

39

Which type of asbestos is most dangerous?

- Rigid, rod like molecule that can poke hole through macrophage

40

What does asbestos lead to?

1. Interstitial Fibrosis
2. Localized fibrotic pleural plaques
3. Malignant mesothelioma
4. Bronchogenic carcinoma
5. Laryngeal Carcinoma
6. Pleural effusion

41

What cause malignant mesothelioma?

Asbestos

42

Hallmark of asbestos?

- Fibrinous "Asbestos bodies"
- Looks like beaded structure with nobby head
- Fe will sit on and coat the asbestos body

43

How much does asbestos increase cancer risk?

- 55x if a smoker
- 5 x if not a smoker
- 1000x increased risk in mesothelioma
***Family members are at risk through indirect exposure
***Can occur 20 - 40 years after exposure

44

Characteristics of Sarcoidosis?

- Multisystem disease of unknown etiology
- Characterized by non caseating granulomas
- Diagnosis of exclusion in which TB or fungus must be ruled out when granulomas are seen

45

What is a granuloma?

- Lymphocytes, macrophages that have fused to form giant cells

46

Clincial presentation of sarcoidosis?

- 90% with bilateral hilar lymphadenopathy or parenchymal abnormality on chest xray

47

What leads to sarcoidosis?

- Genetic abnormality with environmental trigger

48

When are asteroid and schaumann bodies seen?

Sarcoidosis

49

Manifestations of sarcoidosis?

- SOB with dry cough
- Fever fatigue sweats
- Eye in inflammation: Uveitis
- Erythema nodosum
- Restrictive cardiomyopathy

50

What is erythema nodosum?

Inflammatory lesions of subcutaneous fat see in skin in sarcoidosis

51

Why is vitamin D hypercalcemia see in sarcoidosis?

- Granulomas produce enzyme increase conversion of Vitamin D to active form to increase Ca absorption