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restrictive lung disease Flashcards

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1
Q

definition of restrictive lung disease

A

characterized by a reduction in lung volume, alteration in lung parenchyma, and a disease of pleural, chest wall, and neuromuscular status

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2
Q

what values are reduced in restrictive lung disease

A

total lung capacity
vital capacity
resting lung volume

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3
Q

what two groups can restrictive lung disease be divided into

A 
lung parenchyma (intrinsic)
extraparenchyma (extrinsic)
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4
Q

what does intrinsic lung parenchyma result in?

A

inflammation or scarring of lung tissue

filling of air spaces with exudates and debris

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5
Q

what does extraparenchyma restrictive lung disease result in?

A

diseases of the chest wall, pleura and respiratory muscles

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6
Q

what condition causes extrinsic restrictive lung disease with external pressure?

A

obesity

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7
Q

types of intrinsic lung disease

A

sarcoidosis
indiopathic pulmonary fibrosis (IPF)
interstitial lung disease
ARDS, IRDS

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8
Q

causes of interstitial lung disease

A

asbestosis, silicosis, pneumoconiosis
medication, drug, radiation
hypersensitivity pneumonitis
rheumatoid arthritis, lupus, scleroderma

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9
Q

causes of extrinsic restrictive lung disease

A

myasthenia gravis
guillain barre
kyphosis and chest wall deformities
dz that restricts lower thoracic or abdomen volume (obesity, hernia,ascities)

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10
Q

what values are reduced in restrictive lung disease?

A

TLC or VC

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11
Q

what values are preserved in restrictive lung disease

A

airflow
resting lung volume
(measured by functional residual capacity (FRC))

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12
Q

what muscles have to contract in order for the lungs to inflate?

A

respiratory muscles
diaphragm
external costal

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13
Q

is inflation or deflation passive?

A

deflation

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14
Q

volume of air in the lungs when respiratory muscles are fully relaxed and no airflow is present

A

FRC (functional residual capacity)

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15
Q

what balances inward elastic recoil of lungs and outward elastic recoil of the chest wall?

A

FRC

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16
Q

what is a multi-system inflammatory disease of unknown etiology (especially lungs, lymph nodes, and skin) that causes intrinsic restrictive lung disease?

A

sarcoidosis

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17
Q

what manifests as non-necrotizing granulomas predominantly in the lungs and intrathoracic lymph nodes and causes an exaggerated immune response

A

sarcoidosis

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18
Q

what cells are part of the inflammatory immune process in sarcoidosis?

A

T cells-WBCs lymphocytes

which start the immune reaction, identify the illness, and share info with other immune cells

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19
Q

what do T cells cause?

A

an exaggerated immune response

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20
Q

who gets sarcoidosis commonly?

A

african americans

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21
Q

age onset of sarcoidosis

A

25-45

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22
Q

how is sarcoidosis in african americans different?

A

more severe and chronic

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23
Q

multiorgan inflammation of sarcoidosis presents on

A

lung (90%)
lymphadenopathy (33%)
skin (25%)

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24
Q

what percent of sarcoidosis is asymptomatic?

A

5%

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25
non-specific symptoms of sarcoidosis?
``` fever weight loss fatigue arthraliga nagging cough ```
26
main complaints of sarcoidosis
``` pulmonary complaints DOE cough chest pain hemoptysis (rare) crackles (pulm infiltrates) mediastinal/hilar nodes ```
27
other signs of sarcoidosis
``` erythema nodosum skin lesions lupus pernio uveitis lacrimal gland granduloma ```
28
CXR staging for sarcoidosis
``` 0-nml 1-b/l hilar lymphadenopathy 2-BHL + infiltrates 3-infiltrates 4-fibrosis ```
29
what do non-caseating grandulomas secrete
vitamin-D like hormone | angiotensin converting enzyme
30
what does the vitamin D like hormone in sarcoidosis cause?
hypercalcemia | hypercalcuria (more common)
31
what is the best imaging for sarcoidosis?
CXR
32
what other imaging could you do for sarcoidosis workup
bronchoscopy transbronchial/transesophageal US guided bx of mediastinal nodes Bx of skin, lymph nodes, lacrimal glands, conjunctiva
33
relative level of VC in pulm function tests in sarcoidosis patient?
down
34
DLCO levels in sarcoidosis pts?
down (<60%)
35
O2 sat for sarcoidosis patients?
<90% with 6 minute walk test
36
what is one of the monst clinically valuable tests for lung function?
DLCO
37
what does DLCO measure
the ability of lungs to transfer gas from inhaled air to the RBC in pulmonary capillaries
38
what can >75% of sarcoidosis patients be treated with?
NSAIDs
39
what is the mainstay of therapy for sarcoidosis?
corticosteroids
40
how do you dose prednisone for sarcoidosis?
QD then tapered over 6 months
41
what are sarcoidosis patients initiated on?
NSAIDS and steroids
42
what do you prescribe for a sarcoidosis patient if they cannot tolerate steroids?
methotrexate
43
what adverse effects does chloroquine have?
cutaneous lesions hypercalcemia neurological bone lesions
44
what stage sarcoidosis patients get lung transplants? and what are their relative VC and FEV1 levels?
stage 4 | <40% FEV1
45
followup for sarcoidosis patients
CXR and PFT q6-12 mo | annual slit lamp eye exam
46
a specific form of chronic, progressive interstitial scarring of unknown cause
IPF (idiopathic pulmonary fibrosis)
47
what sex is more prone to getting IPF
men
48
what age group is most prone to IPF
>50
49
what is the prognosis for IPF
2-5 years post diagnosis
50
risk factors for IPF
``` genetics viruses dust exposure GERD smoking ```
51
what disease is IPF thought to be?
epithelial-fibroblastic disease
52
what does some unknown insult disrupt in IPF
alveolar epithelial cells causing diffuse epithelial cell disorganization
53
why isn't IPF caught sooner?
most DOE patients are referred to a cardilogist 5% are asymptomatic weak history must exclude any other causes obstructive sleep apnea in 88% of patients
54
non-specific IPF symptoms
``` exertional dyspnea (insidious and progressive) non-productive cough ```
55
systemic IPF symptoms
weight loss low-grade fever fatigue arthralgia/myalgia
56
IPF PE findings
fine inspiratory crackles digital clubbing PH
57
symptoms of PH
right ventricular heave Increased JVP pedal edema tricuspid regurgitation
58
IPF diagnostic testing
``` lung Bx decreased DLCO elevated ANA, RF, CRP, ESR CXR CT ```
59
what is seen on a CXR of an IPF pt?
reticular opacities at lung bases | honeycombing
60
what is seen on a high-res CT of an IPF pt?
patchy reticular opacities
61
comorbid conditions with IPF
``` COPD OSA GERD CAD smoking ```
62
what vaccines should IPF patients get?
influenza | pneumococcal
63
when should an IPF patient get O2 therapy?
when pO2 <88%

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