Restrictive d/o Flashcards

1
Q

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

A

COMMON!

chronic, progressive interstitial fibrosis caused by inflammation

happens to lung parenchyma

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2
Q

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

A

Prevents alveoli from inflating - leads to shunting

Capillaries get occluded by fibrosis - leads to dead space

Capillary membrane thickens - leads to decreased diffusion

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3
Q

What are the signs and sx of idiopathic pulmonary fibrosis?

A

gradual dyspnea, non-productive cough

inspiratory crackles at lung bases, clubbing of fingers

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4
Q

How will you dx idiopathic pulmonary fibrosis?

A

Bx: honeycombing

CXR/CT: honeycombing, ground glass opacities

PFT: normal or high (restrictive pattern)

DLCO: decreased

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5
Q

How do you treat idiopathic pulmonary fibrosis?

A

lung transplant

manage by smoking cessation, 02

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6
Q

What is hypersensitivity (HS) pneumonitis?

A

lung inflammation affecting alveoli and bronchioles;

caused by exposure to dusts, molds, chemicals

Type IV HS reaction (cell-mediated)

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7
Q

What are some examples of HS pneumonitis?

A

Farmer’s lung (moldy hay)

Bird Breeder’s lung (bird feces, feathers)

Chemical Worker’s lung (plastics, polyurethane manufacturing)

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8
Q

What are the symptoms of acute HS pneumonitis?

A

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

inspiratory crackles

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9
Q

What are the sx of subacute HS pneumonitis?

A

intermittent and gradual

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

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10
Q

What are the sx of chronic HS pneumonitis?

A

NO hx of acute episodes

progressively worsening dyspnea, wt loss, clubbing, tachypnea

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11
Q

What will you see with biopsy of HS pneumonitis?

A

granulomas, less organized in acute phase

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12
Q

How do you dx HS pneumonitis?

A

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

CT: ground glass appearance

PFT: normal or high (restrictive pattern)

DCLO: decreased

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13
Q

How do you treat HS pneumonitis?

A

avoid antigen

corticosteroids

and wish them good luck w/ chronic

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14
Q

What is pneumoconiosis?

A

chronic lung disease due to inhaled particulate matter

chronic (parenchymal) fibrotic lung disease

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15
Q

What are examples of pneumoconiosis?

A

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

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16
Q

What is silicosis?

A

exposure to silica/quartz, seen in miners

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17
Q

What are the S/S of silicosis?

A

asymptomatic

dyspnea on exertion, nonprod cough

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18
Q

How do you dx silicosis?

A

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

19
Q

What does silicosis put a pt at increased risk for?

A

2ndary infections like TB and CT disorders

20
Q

What is coal worker’s pneumoconiosis (cwp)?

A

inhalation of coal dust

21
Q

What are the s/s of cwp?

A

pattern of COPD - worse with smokers

22
Q

How do you dx cwp?

A

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

23
Q

What is berylliosis?

A

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

24
Q

What are s/s of berylliosis?

A

similar to silicosis (DOE, nonprod cough)

w/ joint pains, wt loss, fever

25
Q

How do you dx berylliosis?

A

beryllium lymphocyte proliferation test

sometimes CXR shows nonspecific abnormalities

Looks like sarcoidosis w/ granulomas, but less lymphadenopathy

26
Q

How do you tx berylliosis?

A

steroids and 02

if those fail: methotrexate

27
Q

What can berylliosis lead to?

A

increased risk of lung, stomach, colon CA

28
Q

What is asbestosis?

A

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

29
Q

What are the s/s of asbestosis?

A

similar to silicosis

(asymptomatic +/- DOE, nonprod cough)

30
Q

How do you dx asbestosis?

A

CXR: pleural plaques, honeycombing, in lower lobes

“shaggy heart sign”

Bx: asbestos bodies (brown rods)

31
Q

How do you tx asbestosis?

A

Supportive mostly; steroids, 02, lung transplant

smoking cessation

32
Q

What does asbestosis put pt at an increased risk for?

A

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

33
Q

What should you enjoy?

A

Every sandwich

34
Q

What is sarcoidosis (pathophys)?

A

increased T cell response –> granulomas in the lungs

granulomas –> fibrosis

lungs MC affected, but also skin, eyes, lymph nodes

35
Q

What causes sarcoidosis?

A

unknown

genetic and environmental factors lead to disordered immune regulation

36
Q

Who gets sarcoidosis?

A

20-40y

higher risk in AA, Northern Europeans, females

37
Q

What are s/s of sarcoidosis?

A

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)

38
Q

How do you dx sarcoidosis?

A

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

39
Q

What will you find on radiographs of sarcoidosis?

A

CXR: bilateral hilar lymphadenopathy, interstitial lung disease pattern

CT high res: LA, nodules, ground glass opacities, infiltrates

can also do Gallium scan

40
Q

What will PFT of sarcoidosis show?

A

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

41
Q

What lab findings will you see with sarcoidosis?

A

increased ACE levels

hypercalcemia/uria

eosinophilia

cutaneous anergy (dec skin activity to common skin antigens)

42
Q

How do you treat sarcoidosis?

A

Many have spontaneous remission w/in 2y

if tx is needed: PO corticosteroids

NSAIDs for MSK sx

43
Q

What is the prognosis for sarcoidosis?

A

good!

40% spontaneously resolve

40% better w/ tx

20% irreversible lung injury