restrictive lung disease Flashcards

1
Q

restricted breathing mechanics or
capacity to diffuse gases through alveoli restricted → ↓ lung volumes, ↑ work of breathing, inadequate ventilation (O2 into alveoli) and/or oxygenation (O2 to blood)

A

restrictive lung disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

hallmark sign of restrictive lung disease

A

↓ lung volume

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

restrictive lung diseases that interfere with breathing mechanics or gas diffusion

A
1) muscular (normal A-a gradient):
myasthenia gravis
GBS
polio
2) structural (normal A-a gradient): restrict ability of lung to expand and volume of air into lung
severe kyphosis
scoliosis
chest wall deformities
obesity
diaphgragmatic hernia
3) interstitial disease (↑A-a gradient, impaired gas diffusion):
ARDS
pneumoconiosis
sarcoidosis
etc.
pneumonia
lung abscesses
lung cancer
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

↑A-a gradient

A

interstitial lung disease

inability to transport O2 from alveoli to pulmonary capillary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

risk factors for acute respiratory distress syndrome (cause severe damage to lungs)

A
shock
infection
toxic gas inhalation
aspiration
high O2 concentration for extended time
pancreatitis
heroin OD
sepsis
trauma
uremia
amniotic fluid embolism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

severe lung damage → inflammatory cells/mediators and O2 free radicals → damage to endothelial cells and alveolar epithelial (type I) cells → diffuse alveolar damage (DAD) → viscous cycle of inflammation hard to control
also hyaline membrane disease (HMD) → inflammation causes protein rich fluid leaks into alveoli to form intra-alveolar hyaline membrane→pulm edema → ↓ diffusion capacity of O2 → hypoxia induced damage to cells
also coagulation cascade activation →DIC

A

acute respiratory distress syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

cause: surfactant deficiency →↑ surface tension → alveolar collapse

A

neonatal respiratory distress syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

when are type II pneumocytes mature in neonate

A

34 wk GA

if delivery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

risk of supplemental O2 in newborn

A

retinopathy of prematurity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

risk factors for neonatal respiratory distress syndrome

A

prematurity
maternal diabetes
Csection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

chronic fibrotic disease of lung due to INHALATION of inorganic dusts
damage due to MACROPHAGES: ingest foreign material → release inflammatory factors →fibrosis

A

pneumoconiosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

inhale carbon: coal dust → alveolar macrophages eat it →accumulate in CT
can be seen in urban dwellers, smokers too
black pigment in lung
ASYMPTOMATIC

A

anthracosis: mild coal workers pneumoconiosis (CWP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

inhale carbon: coal dust

small fibrotic lung nodules - UPPER LOBES

A

simple coal workers pneumoconiosis (CWP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

inhale carbon: coal dust

progressive massive fibrosis - UPPER LOBES

A

complicated coal workers pneumoconiosis (CWP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

inhale asbestos: shipbuilding, roofing, pipe fitting, plumbing (SPOUSE doing laundry from worker may get asbestosis)
inhaled asbestos → LOWER lung or mesothelial layers → macrophages try to clear toxins → chronic inflammation →
localized pleural plaques
interstitial fibrosis
recurrent pleural effusion

A

asbestosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

gerruginous bodies: golden brown fusiform rods (look like dumbells)

A

asbestos fibers are coated with iron containing protein material

17
Q

↑ risk mesothelioma, laryngeal cancer, and lung cancer (squamous cell + adenocarcinoma)
if smoker: greater risk of lung cancer

A

asbestosis

18
Q

worker in mining, demolition on concrete, stonecutting, SANDBLASTING
inhaled → macrophages try to clear it → chronic inflammation → fibrosis
UPPER LOBES

A

silicosis

19
Q

coal miner is at risk for

A

coal dust: coal workers’ pneumoconiosis (UPPER)
silica: silicosis (UPPER)
radon

20
Q

“eggshell” calcification of hilar lymph nodes

A

silicosis

21
Q

↑ susceptibility to TB

A

silicosis: (silicotuberculosis)

silica crystals inhibit macrophages from killing phagocytosed mycobacteria

22
Q

complication of silicosis

A

↑ risk TB

2x risk of lung cancer

23
Q

high-tech electronics: aerospace mannufacturing
cell-mediated immunity → NONCASEATING granuloma (looks like sarcoidosis)
↑ risk lung cancer

A

berylliosis

24
Q

lung diseases with noncaseating granuloma

A

berylliosis

sarcoidosis

25
Q

bilateral hilar adenopathy

A

sarcoidosis

26
Q

occurs if genetic predisposition to ineffective repair of alveolar epithelial cells injured by environemental exposures
2 genetic mutations: telomerase, mucin MUC5B
> 50 yo, survival 3 years
patchy interstitial fibrosis

A

idiopathic pulmonary fibrosis (cryptogenic fibrosing alveoloitis or usual interstitial pneumonia)

27
Q

honeycomb lung on CT

A

idiopathic pulmonary fibrosis:

cystic spaces

28
Q

focal collections of Langerhan cells accompanied by eosinophilic granulomas
young adult smoker
treatment: stop smoking and improve

A

pulmonary langerhan cell histiocytosis

29
Q

Birbeck granules: tennis racket shaped cyto organelles

A

pulmonary langerhan cell histiocytosis:

in Langerhan cells

30
Q

anti-basement membrane antibodies

A

goodpasture syndrome

31
Q

interstitial lung disease + kidney disease (glomerulonephritis)

A

goodpasture syndrome
or
granulomatosis with polyangitis (upper airway disease)

32
Q

triad:
necrotizing granulomas of UPPER and/or LOWER respiratory tract
necrotizing or granulomatous vasculitis mostly in LUNGS + UPPER airways
focal necrotizing glomerulonephritis

A

granulomatosis with polyangitis (Wegener)

33
Q
inhalation of organic dusts → inflammation of alveoli
examples:
farmers lung: inhale moldy hay
feathers, feces of birds
mushroom
A

hypersensitivity pneumonitis

34
Q

restrictive lung disease due to medications

A

bleomycin: pneumonitis with infiltrates >70 yo
busulfan: acute lung injury, chronic interstitial fibrosis, alveolar hemorrhage
amiodarone: fatal pulmonary fibrosis (check TFT, LFT, and PFT!)
methotrexate: hypersensitivity-lung lung RXN
radiation: radiation pneumonitis

35
Q

“ground glass” appearance on CXR of infant

A

neonatal respiratory distress syndrome:

interstitial opacities