Restrictive d/o

Question 1

What is idiopathic fibrosing interstitial pneumonia (AKA idiopathic pulmonary fibrosis)?

Answer 1

COMMON!

chronic, progressive interstitial fibrosis caused by inflammation

happens to lung parenchyma

Question 2

What occurs in the lungs as a result of fibrosis in idiopathic pulmonary fibrosis?

Answer 2

Prevents alveoli from inflating - leads to shunting

Capillaries get occluded by fibrosis - leads to dead space

Capillary membrane thickens - leads to decreased diffusion

Question 3

What are the signs and sx of idiopathic pulmonary fibrosis?

Answer 3

gradual dyspnea, non-productive cough

inspiratory crackles at lung bases, clubbing of fingers

Question 4

How will you dx idiopathic pulmonary fibrosis?

Answer 4

Bx: honeycombing

CXR/CT: honeycombing, ground glass opacities

PFT: normal or high (restrictive pattern)

DLCO: decreased

Question 5

How do you treat idiopathic pulmonary fibrosis?

Answer 5

lung transplant

manage by smoking cessation, 02

Question 6

What is hypersensitivity (HS) pneumonitis?

Answer 6

lung inflammation affecting alveoli and bronchioles;

caused by exposure to dusts, molds, chemicals

Type IV HS reaction (cell-mediated)

Question 7

What are some examples of HS pneumonitis?

Answer 7

Farmer’s lung (moldy hay)

Bird Breeder’s lung (bird feces, feathers)

Chemical Worker’s lung (plastics, polyurethane manufacturing)

Question 8

What are the symptoms of acute HS pneumonitis?

Answer 8

Rapid - flulike w/ dyspnea 6-8h post exposure; resolving

inspiratory crackles

Question 9

What are the sx of subacute HS pneumonitis?

Answer 9

intermittent and gradual

dyspnea, productive cough, anorexia, wt loss, pleuritis without fever and chills

Question 10

What are the sx of chronic HS pneumonitis?

Answer 10

NO hx of acute episodes

progressively worsening dyspnea, wt loss, clubbing, tachypnea

Question 11

What will you see with biopsy of HS pneumonitis?

Answer 11

granulomas, less organized in acute phase

Question 12

How do you dx HS pneumonitis?

Answer 12

CXR: diffuse interstitial pattern, opacities in lower lung field, apices spared

CT: ground glass appearance

PFT: normal or high (restrictive pattern)

DCLO: decreased

Question 13

How do you treat HS pneumonitis?

Answer 13

avoid antigen

corticosteroids

and wish them good luck w/ chronic

Question 14

What is pneumoconiosis?

Answer 14

chronic lung disease due to inhaled particulate matter

chronic (parenchymal) fibrotic lung disease

Question 15

What are examples of pneumoconiosis?

Answer 15

Silicosis, coal worker’s pneumoconiosis (CWP), berylliosis, and asbestosis

Question 16

What is silicosis?

Answer 16

exposure to silica/quartz, seen in miners

Question 17

What are the S/S of silicosis?

Answer 17

asymptomatic

dyspnea on exertion, nonprod cough

Question 18

How do you dx silicosis?

Answer 18

CXR - nodules, lymphadenopathy or “eggshell calcifications”, fibrosis in upper lobes

Question 19

What does silicosis put a pt at increased risk for?

Answer 19

2ndary infections like TB and CT disorders

Question 20

What is coal worker’s pneumoconiosis (cwp)?

Answer 20

inhalation of coal dust

Question 21

What are the s/s of cwp?

Answer 21

pattern of COPD - worse with smokers

Question 22

How do you dx cwp?

Answer 22

cxr: small upper lobe nodules, hyperinflation of lower lobes (looks like emphysema)

Question 23

What is berylliosis?

Answer 23

inhalation of nuclear reactors like electronics, manufacturing places, light bulbs

Question 24

What are s/s of berylliosis?

Answer 24

similar to silicosis (DOE, nonprod cough)

w/ joint pains, wt loss, fever

Question 25

How do you dx berylliosis?

Answer 25

beryllium lymphocyte proliferation test sometimes CXR shows nonspecific abnormalities **Looks like** sarcoidosis w/ granulomas, but less lymphadenopathy

Question 26

How do you tx berylliosis?

Answer 26

steroids and 02 if those fail: methotrexate

Question 27

What can berylliosis lead to?

Answer 27

increased risk of lung, stomach, colon CA

Question 28

What is asbestosis?

Answer 28

LT inhalation of asbestos; seen 15-20y post exposure

Question 29

What are the s/s of asbestosis?

Answer 29

similar to silicosis | (asymptomatic +/- DOE, nonprod cough)

Question 30

How do you dx asbestosis?

Answer 30

CXR: **pleural plaques**, honeycombing, in **lower lobes** "shaggy heart sign" Bx: asbestos bodies (brown rods)

Question 31

How do you tx asbestosis?

Answer 31

Supportive mostly; steroids, 02, lung transplant smoking cessation

Question 32

What does asbestosis put pt at an increased risk for?

Answer 32

mesothelioma (most common pleural CA), carcinoma of bronchioles, TB

Question 33

What should you enjoy?

Answer 33

Every sandwich

Question 34

What is sarcoidosis (pathophys)?

Answer 34

increased T cell response --\> granulomas in the lungs granulomas --\> fibrosis lungs MC affected, but also skin, eyes, lymph nodes

Question 35

What causes sarcoidosis?

Answer 35

unknown genetic and environmental factors lead to disordered immune regulation

Question 36

Who gets sarcoidosis?

Answer 36

20-40y higher risk in AA, Northern Europeans, females

Question 37

What are s/s of sarcoidosis?

Answer 37

**1/2 are asymptomatic** and found incidentally pulm: **nonprod cough, dyspnea, chest pain** lymphadenopathy skin: erythema nodosa, ***lupus pernio** (raised discoloration of nose, ear, chin, cheek) - pathognomonic,* maculopapular rash, nodules, **parotid** enlargement **visual**: uveitis, conjunctivitis, cataracts/glaucoma heart: arrhythmias rheum: arthralgias, fever, malaise, wt loss, hepatosplenomegaly neuro: CN palsies, lesions (So basically, pt enters room. Differential: sarcoidosis)

Question 38

How do you dx sarcoidosis?

Answer 38

1) compatible clinical/radiologic findings 2) presence of **noncaseating granulomas** on bx 3) exclusion of other diseases

Question 39

What will you find on radiographs of sarcoidosis?

Answer 39

CXR: **bilateral hilar lymphadenopathy, interstitial lung disease** pattern CT high res: LA, nodules, ground glass opacities, infiltrates can also do Gallium scan

Question 40

What will PFT of sarcoidosis show?

Answer 40

PFT **restrictive** in advanced, but can be normal or obstructive pattern

Question 41

What lab findings will you see with sarcoidosis?

Answer 41

increased ACE levels hypercalcemia/uria eosinophilia cutaneous anergy (dec skin activity to common skin antigens)

Question 42

How do you treat sarcoidosis?

Answer 42

Many have spontaneous remission w/in 2y if tx is needed: PO corticosteroids NSAIDs for MSK sx

Question 43

What is the prognosis for sarcoidosis?

Answer 43

good! 40% spontaneously resolve 40% better w/ tx 20% irreversible lung injury