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Clinical Medicine II > Restrictive Lung Disease > Flashcards

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
what is the FEV1/FVC ratio?
-identification of airflow obstruction | < 70% predicted
26
flow volume loop indications?
Stridor – indication of some type of obstruction Unexplained dyspnea – great indication to do a PFT
27
what maneuver do flow volume loops include?
includes forced inspiratory and expiratory maneuver
28
what does the normal vs obstructive pattern flow volume loop look like?
see less volume in obstruction; normal predictive value outlines what the pts value is
29
what does the normal vs restrictive flow volume loop look like?
-total volume is less
30
what does the diffusing capacity of carbon monoxide (DLCO) measure?
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)
31
what does DLCO help us directly isolate?
Helps us to directly isolate how well gases are diffusing across the pulmonary interstitium
32
what is one of the greatest diagnostic benefits of DLCO?
differentiate the etiology of restrictive lung disease
33
how does DLCO differentiate the etiology of restrictive lung disease?
If DLCO is low, may be due to interstitial lung disease If DCLO is normal, may be due to extrathoracic cause of restriction-obesity, chest wall disorder, neuromuscular disorder -just b/c you have normal DLCO, doesn’t mean you can r/o restrictive lung disease
34
what is restrictive pulmonary disease?
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), restrictive disease are associated with a decreased total lung capacity (HALLMARK)**
35
what are restrictive lung diseases associated with?
decreased total lung capacity (HALLMARK)** | obstructive lung disease - COPD/asthma, are not
36
what 2 groups are restrictive pulmonary diseases divided into?
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
37
what are intrinsic restrictive pulmonary diseases?
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
38
what is medication induced interstitial lung disease?
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
39
what 3 medications cause pulmonary toxicity?
- Amiodarone - Methotrexate - Nitrofurantoin *must be taken chronically for chronic diseases (UTIs, afib, arthritis cancer) to cause pulm tox
40
what is the most common daignosis among patients with interstitial lung disease?
idiopathic fibrosing interstitial pneumonia
41
who is idiopathic fibrosing interstitial pneumonia most common in?
men > 50 y/o
42
what is the prognosis for idiopathic fibrosing interstitial pneumonia?
poor prognosis (mean survival 2-5 years from time of dx)
43
what kind of process is idiopathic interstitial pneumonia?
more of a fibrotic process than inflammatory
44
what is idiopathic fibrosing interstitial pneumonia caused by?
repeated injury to structure or function of the alveolar epithelial cells
45
risk factors for idiopathic fibrosing interstitial pneumonia?
- Smoking - Occupational exposure (stone, metal, wood, organic dusts) - GERD (micro-aspiration)
46
idiopathic fibrosing interstitial pneumonia clinical features (sx's/exam)
Symptoms include: insidious dry cough for months, exertional dyspnea, fatigue, tachypnea Examination: clubbing and inspiratory rales (crackles)
47
what is clubbing?
Nail bed angle becomes smooth and tips of fingers become bulbous IRREVERSIBLE Common in pulm diseases: -cystic fibrosis, AV fistula, idiopathic pulm fibrosis, asbestosis, malignancies of the lung & pleura Also seen with GI disease clubbing can occur w/out an underlying disease
48
is clubbing reversible or irreversible?
IRREVERSIBLE
49
what pulmonary diseases is clubbing seen in?
``` -Cystic fibrosis -AV fistula -Idiopathic pulmonary fibrosis -Asbestosis -Malignancies of the lung and pleura ```
50
what do the PFTs show with idiopathic fibrosing interstitial pneumonia?
-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
what are the most common findings of idiopathic fibrosing interstitial pneumonia on a CXR?
reticular markings (seen in both IPF, CHF) -indicative of some type of fibrotic process of the lungs
52
what do the CT scans show for IPF?
shows diffuse patchy fibrosis with pleural based honeycombing
53
IPF diagnosis
Can be made on the basis of a characteristic presentation (symptomatology in combination with CT imaging) -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
54
what is most definitive for dx of IPF and r/o of other causes?
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
55
what are the 3 txt options for IPF?
- supportive care - medications - surgery
56
IPF and supportive care txt
- Supplemental home oxygen (if not already on it) - Vaccinations (influenza, pneumococcal) - Outpatient Pulmonary Rehab Programs
57
IPF and medication txt
both inhibit parts of the disease pathway -Nintedanib: a tyrosine kinase inhibitor -Pirfenidone (Esbriet): an anti-fibrotic drug (slows down progression of the disease)
58
IPF and surgery txt
Lung transplantation – not a simple process, wait list is long, eligibility is long -Age < 65 y/o -Free of substance abuse (smoking, drugs) - can be a past smoker, but not currently smoking -Acceptable BMI range of 20-29
59
what is pneumoconioses?
“Occupational lung disease” Group of interstitial lung diseases caused by the inhalation and deposition of inorganic particles and mineral dust with subsequent reaction of the lung Include: -Coal worker’s pneumoconiosis -Silicosis -Asbestosis
60
what is coal worker's pneumoconioses caused by?
Caused by prolonged exposure to coal dust, which is inert and cannot be removed by the body - 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
what is the milder form of coal worker's pneumoconioses?
Anthracosis
62
what is another name for coal worker's pneumoconioses?
black lung disease
63
do pts have symptoms in early stages of black lung disease?
early in the disease, pts are usually asymptomatic
64
when do sx's for black lung disease develop and what are they?
symptoms such as chronic cough, fever, and dyspnea on exertion usually developed 10-15 years after exposure
65
imaging and coal worker's pneumoconioses
Radiographically, they may have small, rounded, nodular opacities with a preference for the upper lobes Eventually, there is a development of larger opacities with progressive massive fibrosis -this stage is irreversible and progresses despite cessation of the exposure
66
at what stage is coal worker's pneumoconioses irreversible?
when there are larger opacities with progressive massive fibrosis -the disease is irreversible and progresses despite cessation of the exposure
67
what is the prognosis like for coal worker's pneumoconioses and is there txt for it?
Prognosis is poor NO TREATMENT
68
what is silicosis?
pulmonary disease caused by inhalation of crystalline silica
69
occupations where silicosis occurs?
mining, masonry, glass manufacturing, foundry work, and sandblasting
70
clinical stages of silicosis?
Acute, Chronic, Accelerated Silicosis
71
clinical presentation of silicosis
Cough, Dyspnea, sometimes fever or pleuritic chest pain
72
evaluation of silicosis
- H&P - Labs (testing for Tb) - PFTs - CXR/CT
73
what do the PFTs show for silicosis?
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
CXR/CT & silicosis
bilateral, diffuse, groundglass opacities (Acute) -small, innumerable, rounded densities (Chronic)
75
clinical dx of silicosis based on what 3 elements?
- History of silica exposure - Chest imaging consistent with silicosis - Absence of any other diagnosis (e.g., no infectious process) Lung biopsy if the diagnosis can’t be made clinically
76
txt for silicosis
- No proven specific therapy - Avoid further exposure and supportive care - Steroid therapy (for acute states) - Lung transplantation
77
associated complications of silicosis
- Mycobacterial infection (Tb) - Aspergillosis (fungal infection of the lung) - Lung cancer - Chronic kidney disease
78
what is asbestosis?
Pneumoconiosis caused by inhalational asbestos fibers
79
asbestosis clinical presentation
Asymptomatic for at least 20-30 years after initial exposure - Dyspnea on exertion - Cough - Weight loss
80
what is seen on physical exam of asbestosis?
- Inspiratory crackles | - Clubbing
81
what do the PFTs show with asbestosis?
Reduced vital capacity and total lung capacity; Low DLCO | -restrictive pattern
82
radiographic imaging of asbestosis (CXR, CCT)
CXR: thickened pleura and calcified pleural plaques CCT: coarse honeycombing (in advanced disease); hazy ground-glass appearance of the peripheral pleural surface
83
dx of asbestosis
-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
txt of asbestosis
- 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) -*Smoking Cessation (THIS IS A MUST!!!)* (increases their risk of developing lung cancer)
85
what is a MUST when treating asbestosis?
SMOKING CESSATION
86
complications of asbestosis?
Mesothelioma -Direct correlation b/w asbestosis and mesothelioma cancer of the pleura surface Txt of mesothelioma: can take out entire lung or peel off tumor from lung
87
what is sarcoidosis?
Multisystem granulomatous disorder of unknown etiology
88
what organs does sarcoidosis mostly involve?
lungs | also involves lymph nodes, eyes, skin, liver, spleen, heart, nervous system
89
what is sarcoidosis characterized by?
Characterized pathologically by the presence of non-caseating granuloma (vs. caseating granuloma = Tb)
90
who is sarcoidosis most common in?
young black women and Northern European whites
91
clinical presentation of sarcoidosis
-Cough (dry, hacking) - #1 (not productive cough) - Progressive, worsening dyspnea - Atypical chest discomfort (almost like pleuritic type chest pain) - Fever/night sweats - Weight loss
92
what is the #1 symptom of sarcoidosis?
Cough (dry, hacking) - (not productive cough)
93
what are non-diagnostic tests for sarcoidosis?
Serum blood tests, ACE levels, ESR are non-diagnostic
94
radiographic imaging (CXR, CT) of sarcoidosis
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
how do you diagnose sarcoidosis?
NEED TISSUE BIOPSY B/C NEED TO R/O LYMPHOMA Endobronchial US guided biopsy (EBUS) -get tissue from lymph node Cervical Mediastinoscopy -go thru sternal notch & get tissue from lymph node VATS Lung biopsy (Video-assisted Thoracoscopic Surgery) -take out sample of lung tissue
96
txt of sarcoidosis
Close observation for asymptomatic patients ~90% of patients are responsive to a tapering course of oral corticosteroids over 4-6 weeks during flare-ups
97
what are restrictive lung diseases characterized by?
reduced lung volumes (low TLC, low FVC, low DLCO)
98
when is lung transplantation a viable option for IPF?
for pts w/end stage IPF

Clinical Medicine II (10 decks)

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