Restrictive Lung Disease Flashcards

1
Q

what is the pulmonary interstitium?

A

Network of tissue that extends throughout both lungs, including alveolar epithelium, basement membrane, pulmonary capillary endothelium

Provides support to the alveoli and capillary beds for <b>gas exchange</b>

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2
Q

In normal people, can you see the pulmonary interstitium on X-rays or CT scans?

A

No!

-in normal people, the pulmonary interstitium is so thin that it can’t be seen on X-rays or CT scans

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3
Q

when can you see the pulmonary interstitium on X-rays or CT scans?

A

when then pulmonary interstitium is abnormal

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4
Q

where does gas exchange occur?

A

in the alveoli across the interstitium

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5
Q

what are pulmonary function tests (PFTs)?

A

Non-invasive tests that measure how well the lungs are expanding and contracting and how efficient the exchange of oxygen and carbon dioxide is b/w the blood and the air within the lungs

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6
Q

what are the different types of PFTs?

A

Lung volumes, Spirometry, Spirometry before and after a bronchodilator, and diffusing capacity for carbon monoxide (DLCO)

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7
Q

what is tidal volume?

A

volume of air moved in and out during each breath (normal is 500ml = .5L of air)

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8
Q

what is vital capacity?

A

maximum volume of air that can be exhaled after a maximal inspiration

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9
Q

what is residual volume?

A

volume of air remaining in the lungs after a maximal expiration

  • always some residual volume and it’s hard to measure
  • bulk of residual volume is in airways - bronchus and trachea
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10
Q

what is total lung capacity?

A

volume of air in the lungs after maximal inspiration (includes residual volume)

Summation of 2 volumes – inspiratory and expiratory reserve volumes

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11
Q

what is the most common type of PFT?

A

spirometry

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12
Q

what does spirometry do?

A

Measures the volume of air exhaled (after a maximal inhalation) at specific time points during a forceful and complete exhalation (patients blow as much as they can for about 6 seconds)

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13
Q

what is a spirometer? types?

A

an instrument for measuring the air

Types: incentive spirometer & peak flow meter

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14
Q

what is an incentive spirometer?

A

effort to get patients lungs expanded to prevent post-op pneumonias

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15
Q

what is a peak flow meter?

A

for asthma & COPD; when have chest tightness/asthma attack, will use it and see how much they can blow out

-When smooth muscles are tightening, peak flow values will be lower than normal

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16
Q

what factors do you include in spirometry?

A

Gender, age, height, race -> all play into factors of how you fit along the “normal scale”

AA’s and Asians usually score less – so race is important to enter

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17
Q

what is a patients PFTs compared again?

A

the pts value is compared against a predictive value

pooled data from “normal” individuals w/no hx of lung disease, resp symptoms, have normal CXR & EKG

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18
Q

what can PFTs (spirometry) help do?

A

Can help diagnose and differentiate b/w obstructive lung disease & restrictive lung disease

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19
Q

what is spirometry an important tool for?

A

Important tool in assessing diseases such as Asthma, COPD, Cystic Fibrosis, Pulmonary Fibrosis

Also helps you determine how well the pts therapy is going (e.g., bronchodilator)

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20
Q

what are the important parameters of a PFT?

A

FVC, FEV1, FEV1/FVC ratio

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21
Q

what is the FVC?

A

maximum amount of air exhaled after a maximal inhalation

Takes into account:
-Normal lung tissue, Thoracic cage, Functional respiratory muscles (diaphragm)
<b>-If low, the problem may be a restrictive disorder</b>

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22
Q

what is the FEV1?

A

amount of air exhaled in the 1st second

  • very important when looking at <b>obstructive disease</b>
  • most people can expel 70% of their vital capacity in one second

Reduced values may indicate “obstructed” or narrowed airways
(e.g., in COPD – can’t blow out as much air as a normal person can, usually < 70%)

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23
Q

what does a low FVC indicate?

A

restrictive lung disorder

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24
Q

what does a low FEV1 indicated?

A

obstructed lung disease or narrowed airways

normal is 70%

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25
Q

what is the FEV1/FVC ratio?

A

-identification of airflow obstruction

< 70% predicted

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26
Q

flow volume loop indications?

A

Stridor – indication of some type of obstruction

Unexplained dyspnea – great indication to do a PFT

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27
Q

what maneuver do flow volume loops include?

A

includes forced inspiratory and expiratory maneuver

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28
Q

what does the normal vs obstructive pattern flow volume loop look like?

A

see less volume in obstruction; normal predictive value outlines what the pts value is

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29
Q

what does the normal vs restrictive flow volume loop look like?

A

-total volume is less

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30
Q

what does the diffusing capacity of carbon monoxide (DLCO) measure?

A

Measures the overall function of the alveolar-capillary membrane

Looks at how well gas exchange is happening

measure of how much CO is left behind after inhaling a known volume/concentration of different gases (including CO)

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31
Q

what does DLCO help us directly isolate?

A

Helps us to directly isolate how well gases are diffusing across the pulmonary interstitium

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32
Q

what is one of the greatest diagnostic benefits of DLCO?

A

differentiate the etiology of <b>restrictive lung disease</b>

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33
Q

how does DLCO differentiate the etiology of restrictive lung disease?

A

If DLCO is low, may be due to <b>interstitial lung disease</b>

If DCLO is normal, may be due to <b>extrathoracic cause of restriction</b>-obesity, chest wall disorder, neuromuscular disorder

-just b/c you have normal DLCO, doesn’t mean you can r/o restrictive lung disease

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34
Q

what is restrictive pulmonary disease?

A

Inability to completely fill their lungs with air

  • lungs are “restricted” from fully expanding
  • characterized by reduced lung volumes

Unlike obstructive lung disease (COPD, Asthma), <b>restrictive disease are associated with a decreased total lung capacity (HALLMARK)**</b>

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35
Q

what are restrictive lung diseases associated with?

A

<b>decreased total lung capacity (HALLMARK)**</b>

obstructive lung disease - COPD/asthma, are not

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36
Q

what 2 groups are restrictive pulmonary diseases divided into?

A

Divided into 2 groups based on anatomical structures:

Intrinsic: diseases of the lung parenchyma, inflammation or scarring of lung tissue

Extrinsic: extra-pulmonary diseases involving the chest wall, pleura, and respiratory muscles
-obesity, myasthenia gravis, ALS, kyphoscoliosis

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37
Q

what are intrinsic restrictive pulmonary diseases?

A

diseases of the lung parenchyma, inflammation or scarring of lung tissue

(1) Idiopathic fibrotic interstitial pneumonia (formerly called Idiopathic pulmonary fibrosis)
(2) Penumoconioses
(3) Sarcoidosis

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38
Q

what is medication induced interstitial lung disease?

A

These meds cause pulmonary toxicity:

  • Amiodarone
  • Methotrexate
  • Nitrofurantoin

These meds cause pulmonary toxicity when pt takes them for a chronic condition (e.g., chronic UTIs, chronic afib, arthritis, cancer)

-If only have 1 dose and develop acute SOB, that’s a hypersensitivity reaction

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39
Q

what 3 medications cause pulmonary toxicity?

A
  • Amiodarone
  • Methotrexate
  • Nitrofurantoin

*must be taken chronically for chronic diseases (UTIs, afib, arthritis cancer) to cause pulm tox

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40
Q

what is the most common daignosis among patients with interstitial lung disease?

A

idiopathic fibrosing interstitial pneumonia

41
Q

who is idiopathic fibrosing interstitial pneumonia most common in?

A

men > 50 y/o

42
Q

what is the prognosis for idiopathic fibrosing interstitial pneumonia?

A

poor prognosis (mean survival 2-5 years from time of dx)

43
Q

what kind of process is idiopathic interstitial pneumonia?

A

more of a fibrotic process than inflammatory

44
Q

what is idiopathic fibrosing interstitial pneumonia caused by?

A

repeated injury to structure or function of the alveolar epithelial cells

45
Q

risk factors for idiopathic fibrosing interstitial pneumonia?

A
  • Smoking
  • Occupational exposure (stone, metal, wood, organic dusts)
  • GERD (micro-aspiration)
46
Q

idiopathic fibrosing interstitial pneumonia clinical features (sx’s/exam)

A

Symptoms include: <b>insidious dry cough for months</b>, exertional dyspnea, fatigue, tachypnea

Examination: <b>clubbing</b> and inspiratory rales (crackles)

47
Q

what is clubbing?

A

Nail bed angle becomes smooth and tips of fingers become bulbous

<b>IRREVERSIBLE</b>

Common in <b><u>pulm diseases</u>:</b>
-<b>cystic fibrosis</b>, AV fistula, <b>idiopathic pulm fibrosis, asbestosis</b>, malignancies of the lung & pleura

Also seen with GI disease

clubbing can occur w/out an underlying disease

48
Q

is clubbing reversible or irreversible?

A

IRREVERSIBLE

49
Q

what pulmonary diseases is clubbing seen in?

A
<b>-Cystic fibrosis</b>
-AV fistula
<b>-Idiopathic pulmonary fibrosis</b>
<b>-Asbestosis</b>
-Malignancies of the lung and pleura
50
Q

what do the PFTs show with idiopathic fibrosing interstitial pneumonia?

A

-Reduced FVC, but normal or elevated FEV1/FVC ratio

-Reduced DLCO
(fibroblasts are scarring the interstitium – making distance b/w air and cap bed wider)

-Impaired 6 min walk

51
Q

what are the most common findings of idiopathic fibrosing interstitial pneumonia on a CXR?

A

<b>reticular markings (seen in both IPF, CHF)</b>

-indicative of some type of fibrotic process of the lungs

52
Q

what do the CT scans show for IPF?

A

shows diffuse patchy fibrosis with pleural based <b>honeycombing</b>

53
Q

IPF diagnosis

A

Can be made on the basis of a characteristic presentation (symptomatology in combination with CT imaging)

<b>-Lung biopsy is usually more definitive and can help rule out other possible causes (pneumonitis, Rheumatic disease, histiocytosis) to confirm presence of interstitial pulmonary fibrosis</b>

54
Q

what is most definitive for dx of IPF and r/o of other causes?

A

<b>Lung biopsy</b>

-is usually more definitive and can help rule out other possible causes (pneumonitis, Rheumatic disease, histiocytosis) <b>to confirm presence of interstitial pulmonary fibrosis</b>

55
Q

what are the 3 txt options for IPF?

A
  • supportive care
  • medications
  • surgery
56
Q

IPF and supportive care txt

A
  • Supplemental home oxygen (if not already on it)
  • Vaccinations (influenza, pneumococcal)
  • Outpatient Pulmonary Rehab Programs
57
Q

IPF and medication txt

A

both inhibit parts of the disease pathway

<b>-Nintedanib:</b> a tyrosine kinase inhibitor
<b>-Pirfenidone (Esbriet):</b> an anti-fibrotic drug (slows down progression of the disease)

58
Q

IPF and surgery txt

A

<b>Lung transplantation</b> – not a simple process, wait list is long, eligibility is long
<b>-Age < 65 y/o</b>
<b>-Free of substance abuse (smoking, drugs)</b> - can be a past smoker, but not currently smoking
<b>-Acceptable BMI range of 20-29</b>

59
Q

what is pneumoconioses?

A

<b>“Occupational lung disease”</b>

Group of <u>interstitial lung diseases</u> caused by the <b>inhalation and deposition of inorganic particles and mineral dust with subsequent reaction of the lung</b>

<u>Include</u>:
<b>-Coal worker’s pneumoconiosis
-Silicosis
-Asbestosis</b>

60
Q

what is coal worker’s pneumoconioses caused by?

A

<b>Caused by prolonged exposure to coal dust, which is inert and cannot be removed by the body</b>

  • Leads to inflammation, fibrosis, and sometimes necrosis
  • Coal dust gets inhaled into the air sacs -> engulfed by macrophages which try to get rid of it, but can’t get rid of the coal dust -> particles become embedded in the interstitium and solidify
61
Q

what is the milder form of coal worker’s pneumoconioses?

A

Anthracosis

62
Q

what is another name for coal worker’s pneumoconioses?

A

black lung disease

63
Q

do pts have symptoms in early stages of black lung disease?

A

early in the disease, pts are usually asymptomatic

64
Q

when do sx’s for black lung disease develop and what are they?

A

symptoms such as chronic cough, fever, and dyspnea on exertion usually developed 10-15 years after exposure

65
Q

imaging and coal worker’s pneumoconioses

A

Radiographically, they may have <b>small, rounded, nodular opacities with a preference for the upper lobes</b>

Eventually, there is a <b>development of larger opacities with progressive massive fibrosis</b>
-this stage is <b><u>irreversible</u></b> and progresses despite cessation of the exposure

66
Q

at what stage is coal worker’s pneumoconioses irreversible?

A

when there are larger opacities with progressive massive fibrosis

<b>-the disease is irreversible and progresses despite cessation of the exposure</b>

67
Q

what is the prognosis like for coal worker’s pneumoconioses and is there txt for it?

A

Prognosis is poor

<b>NO TREATMENT</b>

68
Q

what is silicosis?

A

pulmonary disease caused by inhalation of crystalline silica

69
Q

occupations where silicosis occurs?

A

mining, masonry, glass manufacturing, foundry work, and sandblasting

70
Q

clinical stages of silicosis?

A

Acute, Chronic, Accelerated Silicosis

71
Q

clinical presentation of silicosis

A

Cough, Dyspnea, sometimes fever or pleuritic chest pain

72
Q

evaluation of silicosis

A
  • H&P
  • Labs (testing for Tb)
  • PFTs
  • CXR/CT
73
Q

what do the PFTs show for silicosis?

A

Decreased FEV1, and DLCO, normal FEV1/FVC ratio

DLCO may be normal but most cases it’s impaired b/c of fibrosis that is happening

74
Q

CXR/CT & silicosis

A

bilateral, diffuse, groundglass opacities (Acute)

-small, innumerable, rounded densities (Chronic)

75
Q

clinical dx of silicosis based on what 3 elements?

A
  • History of silica exposure
  • Chest imaging consistent with silicosis
  • Absence of any other diagnosis (e.g., no infectious process)

<b><i>Lung biopsy if the diagnosis can’t be made clinically</i><b></b></b>

76
Q

txt for silicosis

A
  • No proven specific therapy
  • Avoid further exposure and supportive care
  • Steroid therapy (for acute states)
  • Lung transplantation
77
Q

associated complications of silicosis

A
  • Mycobacterial infection (Tb)
  • Aspergillosis (fungal infection of the lung)
  • Lung cancer
  • Chronic kidney disease
78
Q

what is asbestosis?

A

Pneumoconiosis caused by inhalational asbestos fibers

79
Q

asbestosis clinical presentation

A

<b>Asymptomatic for at least 20-30 years after initial exposure</b>

  • Dyspnea on exertion
  • Cough
  • Weight loss
80
Q

what is seen on physical exam of asbestosis?

A
  • Inspiratory crackles

- Clubbing

81
Q

what do the PFTs show with asbestosis?

A

Reduced vital capacity and total lung capacity; Low DLCO

<b><i>-restrictive pattern</i></b>

82
Q

radiographic imaging of asbestosis (CXR, CCT)

A

CXR: thickened pleura and calcified pleural plaques

CCT: coarse honeycombing (in advanced disease); hazy ground-glass appearance of the peripheral pleural surface

83
Q

dx of asbestosis

A

-History of asbestos exposure
-Chest imaging consistent with Asbestosis
-Absence of any other diagnosis
-Bronchoalveolar lavage (limited value)
(limited b/c asbestosis is in a dormant phase for 20-30 years & it goes undetected -> damage has already been done)

84
Q

txt of asbestosis

A
  • No proven specific therapy
  • Avoid further exposure and supportive care
  • Steroid therapy (questionable b/c pleural deposits don’t get any benefit from the steroids)

<b>-Smoking Cessation (THIS IS A MUST!!!)
(increases their risk of developing lung cancer)</b>

85
Q

what is a MUST when treating asbestosis?

A

SMOKING CESSATION

86
Q

complications of asbestosis?

A

<b>Mesothelioma</b>
-Direct correlation b/w asbestosis and mesothelioma cancer of the pleura surface

<u>Txt of mesothelioma</u>: can take out entire lung or peel off tumor from lung

87
Q

what is sarcoidosis?

A

Multisystem granulomatous disorder of unknown etiology

88
Q

what organs does sarcoidosis mostly involve?

A

<b>lungs</b>

also involves lymph nodes, eyes, skin, liver, spleen, heart, nervous system

89
Q

what is sarcoidosis characterized by?

A

Characterized pathologically by the presence of <b><u>non-caseating granuloma</u></b>

(vs. caseating granuloma = Tb)

90
Q

who is sarcoidosis most common in?

A

<b>young black women</b> and Northern European whites

91
Q

clinical presentation of sarcoidosis

A

<b>-Cough (dry, hacking) - #1 (not productive cough)</b>

  • Progressive, worsening dyspnea
  • Atypical chest discomfort (almost like pleuritic type chest pain)
  • Fever/night sweats
  • Weight loss
92
Q

what is the #1 symptom of sarcoidosis?

A

Cough (dry, hacking) - (not productive cough)

93
Q

what are non-diagnostic tests for sarcoidosis?

A

Serum blood tests, ACE levels, ESR are non-diagnostic

94
Q

radiographic imaging (CXR, CT) of sarcoidosis

A

CXR: bilateral hilar adenopathy
-L/R hilum -> enlarged lymph nodes in hilum is concerning

CT: right paratracheal lymphadenopathy along with bilateral diffuse reticular infiltrates

95
Q

how do you diagnose sarcoidosis?

A

<b>NEED TISSUE BIOPSY B/C NEED TO R/O LYMPHOMA</b>

<b>Endobronchial US guided biopsy (EBUS)</b>
-get tissue from lymph node

<b>Cervical Mediastinoscopy</b>
-go thru sternal notch & get tissue from lymph node

<b>VATS Lung biopsy (Video-assisted Thoracoscopic Surgery)</b>
-take out sample of lung tissue

96
Q

txt of sarcoidosis

A

<b>Close observation</b> for asymptomatic patients

~90% of patients are responsive to a <b>tapering course of oral corticosteroids over 4-6 weeks during flare-ups</b>

97
Q

what are restrictive lung diseases characterized by?

A

reduced lung volumes (low TLC, low FVC, low DLCO)

98
Q

when is lung transplantation a viable option for IPF?

A

for pts w/end stage IPF