Pathology Test 4 Lung Two- Restrictive Disease

Front 1

What microscopic structure is involved in restrictive lung diseases?

Back 1

this has damage to BM of endothelial and epithelial cells

Front 2

What are the common clinical features of restrictive lung disease?

Back 2

FVC is decreased

FEV: FVC ratios are normal (the lung is smaller, but everything works as it should)

Abnormal ventilation to perfusion ratio---HYPOXIA*

Dyspenia, tachypnea, END inspiratory crackles

Front 3

What are the two CXR pattern to recognize for Diffuse intersitial diseases?

Back 3

Ground Glass pattern (think frosted glass areas)

Reticulonodular pattern- small nodes with irregular lines

Honeycomb lung- advanced

Front 4

What happens to the alveolar septae in intersisital fibrosis?

Back 4

they become seriously thickened** and FIBROUS

Front 5

What should the blood gasses be like for a person with intersitial lung disease?

Back 5

Low pO2
Low O2 sat%

Front 6

What is the morphology of Idiopathic Pulmonary Fibrosis?***

Back 6

this is a the clinical symptoms, with the morphology of usual interstitial pneumonia (peppo!)

On biopsy: Fibroblasts, Hyperplastic type II pneumoctes
On CXR: honeycombing

Cobble stone pleural surface***

Front 7

What chemical mediators of Idiopathic pulmonary fibrosis cause disease?

Back 7

Inflamamtion's Th2, IL-4, IL-13

TGF-Beta-1

Front 8

What is TGF-B1's role in Idiopathic pulmonary fibrosis?

Back 8

TGF-B1 is released from injured type 1 pneumocytes, this transforms fibroblasts into myofibroblasts

inhibits telomerase, causes apoptosis of pneumocytes

Front 9

What is the clinical course of IPF? Signs?

Back 9

40-70 years old
Gradual progression
Dry or Velcro crackles on inspiration
Hypoxia

Front 10

What does Caveolin do?

Back 10

this usually inhibits fibrosis, but it is taken out by TGF-B1 in IPF

Front 11

What is the clinical behavior of COP? (cryptogenic organizing pneumonia)

Back 11

cough, dyspenia,
subpleural or peribronchial patchy airspace consolidation*

Front 12

What four vascular diseases are associated with pulmonary fibrosis?

Back 12

SLE
Rhemuatoid
Scleroderma
Dermatomyositis

Front 13

What causes coal workers pneumoconiosis?

Back 13

being derek zoolander for a day "i think ive got the black lung pop!"

or carbon (not coal) dust

Front 14

Do people with black lung have increased risks of cancer or TB?

Back 14

Nope they do not

Front 15

What three diseases can you get from Black lung, and how bad are they?

Back 15

Anthracosis- no problems

Simple coal workers pneumoconiosis- little/no dysfunction, less than 10% progress

Complicated coal workers pneumoconiosis- respiratory compromise

Front 16

What is Anthracosis?

Back 16

accumulation of black carbon pigment in the lungs.

causes my macrophages taking up junk, and migrating to perilymphatic lung, pleura, and hilar lymph nodes

Front 17

What is Caplans syndrome?

Back 17

Lung disease of Rheumatoid arthritis AND pneumoconiosis at the same time!

progresses rapidly

Front 18

What is the most common chronic occupational disease...in the world?

Back 18

silicosis

Front 19

What form of silica is the worst?

Back 19

crystalline form-quartz

Front 20

How does silicosis damage your lungs?

Back 20

SiOH are rich in free radicals,
these bind with epithelial cell membrane phospholipids. causing damage.

Macrophages that have ingested silica are activated and release TNF/IL-1 to cause fibrosis

Front 21

What are the clinical features of chronic silicosis?

Back 21

NODULES in upper lobes (because its inhaled, yah?)
These may cavitate

Progressive Massive Fibrosis (scars up to 10cm in diameter)- which causes dyspnea

Calcification

polarized birefrigent material in nodules. (has two different kinds of refraction)

Front 22

If you have silicosis, what are you at increased risk for?**

Back 22

TB!!

Caplan's syndrome

(but not cancer)

Front 23

What are the five types of asbestos related disease?

Back 23

Asbestosis (fibrosis)
Pleural plaques
Pleural effusions
Bronchogenic carcinoma
Mesothelioma

Front 24

What types of asbestos are most associated with mesothelioma?

Back 24

Amphiboles
straight, brittle, insoluble fibers

Front 25

How does asbestos causes disease?

Back 25

Fibers generate free radicals, absorb and hold toxic chemicals

Macrophages try and eat these fibers, and become Activated**, which then release fibrogenic factors**

Front 26

What are the rod forms of asbestos in the lungs called? what do they look like?

Back 26

Ferruginous bodies

-golden-brown, fuiform, or breaded rods
coated by iron containing protein

Front 27

What is the most common manifestation of Asbestos?

Back 27

Pleural Plaques

Front 28

What does the patter of diffuse pulmonary fibrosis due to asbestos look like?

Back 28

Honeycomb

Front 29

What two cancers have a high association with asbestos?

Back 29

bronchogenic carcinoma

mesothelioma

Front 30

What three drugs/therapies are associated with Pulmonary fibrosis

Back 30

Bleomycin, Busulfan *** (have been in two lectures)

Radiation- (looks like ARDS, up to 6 months post rads Tx)

Front 31

What is sarcidosis?

Back 31

this is multisystem disease, of UKO.

causes noncaseating (hard) granulomas in many tissues

Front 32

What are the main signs of Sarcoidosis on Xray?

Back 32

Bilateral hilar lymphadenopathy***

, or lung involvement.

Pulmonary infiltrates 1-2cm granulomas producing restrictive lung disease

Front 33

DO NOTE the difference between silicosis, and sarcoidosis in the lungs- they both have round spots in the lung...

Back 33

BUT silicosis is collagenous, and doesnt have cells (nuceli) in the center of it

ANd Sarcoidosis is a typical granuloma, giant cells, neutrophils and all that goodness in the granuloma

Front 34

What is the typical pt with sarcoidosis?

Back 34

black woman, 20-40, in southeastern USA

Front 35

What is the immunologic basis of sarcoidosis?

Back 35

Type IV cell mediated immune response to unknown antigen

Front 36

What systemic immunologic abnormalities are found in sarcoidosis?

Back 36

Anergy to skin test antigens

polyclonal hypergammaglobulinemia

Front 37

What are asteroid bodies?

Back 37

these are stellate structures within giant cells, found in sarcoidosis

Front 38

what are schaumann bodies?

Back 38

these are concretions that are found in sarcoidosis

Front 39

What are the skin lesions associated with sarcoidosis?

Back 39

Skin plaques and nodules

Lupus perino- violaceous, scaling plaques

Erythema nodosum- a hypersensitivity vasculitis

Front 40

Because Sarcoidosis is usually a Dx of exclusion, what should you rule out?

Back 40

other causes of granulomas, like TB and fungus

Front 41

What are the LAB tests for sarcoidosis?

Back 41

lymphocytoepnia;s
anemia
hypercalcemia
polyclonal gammapathy
HIGH ACE (angiotensin converting enzyme) for active sarcoidosis

Front 42

What causes hypersensitivity pneumonitis?

Back 42

immunologically mediated intersitial lung disorders.

causes by Type III hypersensitivity

Front 43

although hypersensitivity pneumonitis is type III, what can it turn into in 2/3rds of cases?

Back 43

this turns into Type IV interstitial NONcaseating granulomas

Front 44

What are the typical signs of hypersensitivity pneumonitis?

Back 44

intersitial pneumonitis
fibrosis
noncaseating granulomas

Sx's occur 4-6 hours after exposure

Front 45

What are the clinical features of hypersensitivity penumonitiis?

Back 45

symptoms 4-6 hours post exposure.
leukocytosis, cough, dyspnea
Diffuse nodular infiltrates on X-ray
Restrictive FEV:FVC

resolves in 24 hours usually

Front 46

What is the morphology and clinical of berylliosis?

Back 46

noncaseating granulomas in pleura, septa, and bronchovasuclar tissue.

cancer risk

Front 47

What are the signs of pulmonary eosinophilia?

Back 47

eosinophilia on CBC

fever, night sweats, abnormal CXR

Front 48

What things can cause pulmonary eosinophilia?

Back 48

parasites
fungi
drug allergies
aserpgillosis
PAD
asthma
hypersensitivity pneumonitis

Front 49

What causes desquamative intersitial pneumonia?
What characterizes it ?

Back 49

smoking related

Smokers macrophages in the alveoli

Front 50

How is DIP treated?

Back 50

this responds well to corticosteroids

Front 51

What causes acquired pulmonary alveolar proteinosis?

Back 51

antibodies to GM-CSF, an autoimmune reaction.

Front 52

What are the clinical signs of Pulmonary alveolar proteinosis?

Back 52

insidious cough onset.
Production of abundant GELATINOUS MATERIAL, sometimes in chunks

(on microscope, looks like hyaline material everywhere....tons of pink crap in every alveoli- PAS positive)

Front 53

What is the Tx for pulmonary alveolar proteinosis?

Back 53

whole lung lavage, flush that crap out

Front 54

how does smoking related to on the job injuries?

Back 54

they are increased in smokers!

Front 55

What is Cotinine?

Back 55

this is the oxidative metabolite of nicotine.
Specific and sensitive for nicotine exposure

Front 56

What are the LAB tests** seen in smokers?

Back 56

INCREASED everything..
glucose
catecholamines/cortisol
FFA's
WBC's
Carcinoembryonic antigen
carboxyhemoglobin
polycythemia