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Block 9 Week 3 > Restrictive Lung Disease-Baker 2 > Flashcards

Restrictive Lung Disease-Baker 2 Flashcards

1
Q

What is this:

characterized by reduced expansion of lung parenchyma and reduced total lung capacity. I.e you cant get air in :)

A

restrictive lung disease

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2
Q

What does restrictive lung disease present with?

A
  • dyspnea
  • tachypnea
  • end-inspiratory crackles
  • cyanosis
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3
Q

What can restrictive lung diseasae progress to?

A

secondary pulm HTN, right heart failure/cor pulmonale

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4
Q

In restrictive lung disease what will the spirometry exam show?

A
  • Decrease TLC
  • Decrease DLCO (tissue destruction)
  • Decrease FVC
  • Normal to Increase FEV1
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5
Q

When thinking about restrictive lung disease you should determine if the problem is in the chest wall or lung parenchymal/chronic interstitial and infiltrative disease.
If it is chest wall, whats the issues that cause this?

A
  • Neuromuscular
  • Obesity
  • pleural disorders
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6
Q

When thinking about restrictive lung disease you should determine if the problem is in the chest wall or caused by damage to lung parenchymal or if there is chronic interstitial and infiltrative diseases causing it.
If its damage to the lung parenchyma or if its a chronic interstitial and infiltrative diseases that are causes the issues, what are things that can cause this?

A
  • fibrosing
  • granulomatous
  • eosinophilic
  • smoking related
  • other
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7
Q

What are the fibrosing diseases?

A
  • Idiopathic Pulmonary Fibrosis
  • Nonspecific Interstitial Pneumonia
  • Cryptogenic Organizing Pneumoinia
    aka: bronchiolitis obliterans organizing pneumonia (BOOP)
  • Lung Disease from Connective Tissue Disorders
  • Pneumoconiosis
  • Drug Reactions
  • Radiation Pneumonitis
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8
Q

What causes the fibrosing diseases?

A

Something is injuring the lung. The lung “reacts” with inflammation and a wound healing reaction. The honeycombing occurs from the dilated distal airway that is amputated by the fibrosis.

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9
Q

If you see a honeycomb appearnce on a lung what is this?

A

IPF (Idiopathic pulmonary fibrosis)

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10
Q

What kind of syndrome is IPF, what does this mean?

A

Is a clinico-pathologic syndrome. Meaning characteristic clinical picture (dyspnea) + X-ray changes + pathologic changes.

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11
Q

The pathologic changes of IPF are referred to as (blank)

A

Usual interstitial pneumonia (UIP)

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12
Q

Where else can you see the UIP pattern?

A

connective tissue diseases, chronic hypersensitivity pneumonia and asbestosis.

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13
Q

What is the honeycombing in IPF? IPF shows diffuse damage but where is the worst damage?

A

cystic spaces

lower lobe

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14
Q

What is the onset like in IPF? When is the onset? What happens later in the disease?

A
  • Insidious onset with dry cough and dyspnea
  • 40-70
  • hypoxemia and clubbing with gradual deterioration, +/- acute exacerabations
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15
Q

How do you treat IPF?

A
  • steroids, immune suppressants

- lung transplantation is possible

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16
Q

What is this:
looks like IPF but does not show interstitial pneumonia pattern that must be present to be UIP or IPF. (biopsies fail to show diagnostic features of any of the other well-characterized ILD)

A

Nonspecific Interstitial Pneumonia

Diffuse ILD of uknown etilogy

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17
Q

What are the 2 types of NSIP (Nonspecific Interstitial Pneumonia)?

A

Cellular

Fibrosing

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18
Q

What type of NSIP is this:
Mild to moderate chronic interstitial inflammation
Uniform or patchy
Occurs in younger and has better outcomes

A

Cellular

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19
Q

What type of NSIP is this:
Diffuse or patchy interstitial fibrosis
No temporal heterogeneity or honeycombing present
Older population and worse outcome

A

Fibrosing

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20
Q

Which has a better prognosis, NSIP or UIP?

A

NSIP!

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21
Q

Sometimes NSIP and UIP histological findings overlap, if you arent sure what the diagnosis is how should you treat it?

A

as if it was UIP because that has the worst prognosis

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22
Q

What does NSIP resent with and what is the onset?

A
  • dyspnea and cough for several months

- 46-55 years

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23
Q

What are the earliest lesions seen in IPF?

Where will you see fibrosis?

A
  • Fibroblastic focus (nodule of spindle cells arranged in a linear fashion)
  • Temporal heterogeneity-(Normal areas alternating with abnormal areas of varying stage)

-Dense subpleural fibrosis resulting in collapse and obliteration of alveolar air spaces

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24
Q

What does the FVC tell you?

A

the total amount of air exhaled during the forced expiratory volume test

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25
Q

What does the PEF tell you?

A

How fast air is coming out

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26
Q

What does FEV tell you?

A

How much air is being expired per second

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27
Q

What does FEF tell you?

A

FEF-> measures air flow halfway through an exhale

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28
Q

What does TLC and RV tell you?

A

TLC-> total amount of air in your lungs after you inhale as deeply as possible
RV-amount of ai left in lungs after having exhaled completely.

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29
Q

What does DLCO tell you?

A

measures the ability fo lungs to transfer gas from inhaled air to RBCs in pulmonary capillaries

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30
Q

What causes IPF?

A

aberrant wound healing leading to fibrosis

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31
Q

What are environmental factors that can lead to IPD? What are some predispositions that can lead to it?
What is non-modifiable risk?

A

Environmental:
smoking, exposure to metal fumes and wood dust, hair-dressers

Predispositions:
Genetic factors which affect pneumocytes

Being over age 50

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32
Q

What will environmental and predisposing factors lead to?

A

persistent epithelial injury-> abberant (abnormal) wound healing-> interstitial fibrosis

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33
Q

What new drug helps reduce IPF by inhibiting the tyrosine kinase receptors for growth factors for fibroblasts?

A

nintedanib

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34
Q

What is the survival rate of IPF?

A

3-year

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35
Q

How does pirfendione work for IFP?

A

inhibits expression of transforming growth factor B1 therefore reducing fibrosis

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36
Q

What is cryptogenic organizing pneumonia (bronchiolitis obliterans organizing pneumonia)?

A
  • presents w/ cough and dyspnea
  • patchy airspace opacities
  • long term changes of fibrinous exudates
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37
Q

What is this:

polypoid plugs of loos organizing CT (Masson bodies)?

A

Cryptogenic Organizing Pneumonia

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38
Q

In cryptogenic organizing pneumonia is there temporal heterogeneity?Interstitial fibrosis?
honeycomb lung?

A

No
No
No

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39
Q

What does the underlying architecture of cryptogenic organizing pneumonia look like?

A

normal!

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40
Q

What do you call cryptogenic organizing pneumonia that has a cause?

A

organizing pneumonia

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41
Q

How can you get organizing pneumonia?

A

do to a secondary condition:

  • 2ndary to infections or inflammatory injury
  • Viral and bacterial pneumonia
  • Inhaled toxins
  • Drugs
  • Connective tissue disease
  • GVHD
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42
Q

What CT disease can cause this:
30-40% of patients
Chronic pleuritis with or without effusion
Diffuse interstitial pneumonitis and fibrosis
Intrapulmonary rheumatoid nodules
Pulmonary hypertension

A

Rheumatoid arthritis

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43
Q

What CT disease can cause this:
Diffuse interstitial fibrosis
-UIP or NSIP, but NSIP is more common

A

Systemic Sclerosis

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44
Q

What CT disease can cause this:
Patchy, transient parenchymal infiltrates
Occasionally severe lupus pneumonitis

A

Systemic Lupus Erythematosus

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45
Q

What is this:

Disease induced by inhalation of organic and inorganic particulates, chemical fumes and vapors

A

Pneumoconioses

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46
Q

What size particles are the most dangerous and why?

A

1-5 uM because they are the perfect size to reach and settle in to the distal airways

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47
Q

What are factors related to the development of pneumoconioses?

A

amout of dust retained, solubility and reactivity, addional effect of other irritants (smoking)

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48
Q

What size particles are most likely to cause acute lung injury?

A

small particles move into tissues/fluids and can reach toxic levels

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49
Q

What size particles are most likely to evoke fibrosing collagenous pneumoconioses?

A

large particles stay within the lung parenchyma

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50
Q

What willl the large particles of pneumoconioses do within the lung parenchyma?

A

evoke fibrosing collagenous pneumoconioses

51
Q

How do the particles that caused pneumoconioses spread throughout the lungs?

A

bloodstream and lymphatics

52
Q

What is the pathogenesis of pneumoconioses?

A

-dust, anthracotic pigment and particles enter lungs-> creates a mucous blanket on top of ciliated respiratory epithelium-> particles get eaten by macrophages -> macrophages release cytokines-> cell injury-> fibroblast proliferation and collagen deposition.

53
Q

What are the products released by macrophages in pneumoconioses?

A

IL-1, TNF, 02 free radicals

54
Q

If silicone enters lungs what will happen to your lungs?

A

create silicotic nodules surrounded by lymphocytes and macrophages and fibroblasts

55
Q

If asbestos enters the lungs what will it look like?

A

Interstitial fibrosis with thickened visceral pleura

56
Q

How does smoking affect pneumoconioses?

What type of particle does smoking make really bad in pneumoconioses?

A

smoking reduces mucociliary clearance

  • less clearance= more retained particulate matter and thus worsens the effect of all inhald dusts
  • asbestos inhalation
57
Q

What are the three major causes of pneumoconioses?

A
  • coal workers’ pneumoconiosis
  • silicosis
  • asbestos-related diseases
58
Q

What is the spectrum of Coal Workers’ Pneumoconiosis?

A
  • anthrocosis
  • simple CWP
  • Complicated CWP
59
Q

How does Coal Wokers’ pneumoconiosis affect lung function?

A

minimal to no decrease in lung function

60
Q

Minority of patients with (blank) develop progressive massive fibrosis

A

Complicated Coal Worker’s Pneumoconiosis

61
Q

What is coal workers pneumoconiosis associated with?

Is there an increased risk of cancer of TB?

A

-emphysema and chronic bronchitis

No

62
Q

What is the most prevalent occupational disease in the world?
What kind of work can cause this?

A

Silicosis

-foundry work, sandblasting, mining, stone cutting

63
Q

What do the lesions of silicosis look like? Does it have an insidious onset or progressive?

A

Nodular, fibrosing lesions

Slowly progressive from decades of exposure

64
Q

In acute silicosis, you will see an abundant (blank) material in alveoli. It is identical to (blank)

A

lipoproteinaceous

alveolar proteinosis

65
Q

What is the most common offender in silicosis? why?

How does it silicosis present early? how about late?

A

quartz
activates macrophages-> fibrogenic

  • tiny nodules early
  • coalesce into hard collagenous scars
66
Q

Is pure quartz or mixed quartz worse?

A

pure quartz

67
Q

On an x-ray, what will silicosis look like?

A

upper zone nodularity

  • usually no SOB, w/ massive fibrosis
  • eggshell calciication in hilar LN
68
Q

Silicosis increases susceptibility to (blank) and is possibly (blank)

A

tuberculosis (possibly immune related)

carcinogenic

69
Q

What can asbestos do to the pleura?

A
  • pleural effusions
  • fibrous plaques
  • diffuse pleural fibrosis
  • mesothelioma
70
Q

What can asbestos do to the pulmonary parenchyma?

A
  • lung carcinoma

- interstitial fibrosis (asbestosis)

71
Q

What are the 2 forms of asbestos?

A

Serpentine (curly)

Amphibole (needle-like)

72
Q

What is the most common type of asbestos used in industry?

Is this the kind that causes pathology?

A

Serpentine (curly)

least likely to cause pathology-tend to get stuck higher up

73
Q

What is the less prevalent kind of asbestos that causes pathology? Why does it cause pathology?

A

Amphibole (needle-like)

because it is able to go deeper

74
Q

What is the only form of asbestos associated with mesothelioma? Where is it commonly found?

A

amphibole

-on the filter of cigarettes

75
Q

How can asbestos cause cancer?

A

asbestos fibers may adsorb toxic chemicals, increasing cancer potential

76
Q

No smoking and asbestos = (blank)X the risk of lung carcinoma.
No smoking and asbestos= (blank)X the risk of mesothelioma
Smoking and asbestos= (blank)X the risk of lung carcinoma
Smoking and asbestos= (blanks)X the risk of mesothelioma

A

5X
1000X
55X
1000X (no change)

77
Q

What is this:

diffuse pulmonary interstitial fibrosis

A

asbestosis

78
Q

What are asbestos bodies? How are they created?

A
  • asbestos fiber coated with Fe containing proteinaceous material
  • when macrophages try to phagocytize the asbestos fibers
79
Q

What are the areas of the lung that asbestosis affects first?

What do you find in asbestosis?

A

lower lung and subpleura areas

  • asbestos bodies
  • diffuse pulmonary interstitial fibrosis
  • eventual honeycombing
  • Similiar to UIP
80
Q

What are the clinical features of asbestosis?

A
  • dyspnea on exertion (early)
  • -> at rest (later)
  • productive cough
  • rare < 10 years after exposure, more common > 20 yrs
81
Q

If you see a fine reticular pattern in lower lobes on an x-ray, what do you think it is?

A

Asbestosis

82
Q

What will the plaques look like in asbestosis and what will they be made up of?

A

well circumscribed, dense collagen and calcium

83
Q

What parts of the lung do the plaques of absestos affect?

A

anterior and posterolateral parietal pleura and common over domes of the diaphragm

84
Q

What can asbestos do to the pleura besides cause plaques?

A

Pleural effusion of serous fluid
Pleural fibrosis (rare)
Mesothelioma

85
Q

What are the granulomatous restrictive diseases?

A

Sarcoidosis

Hypersensitvity Pneumonitis

86
Q

What is this:

a systemic disease of unknown cause filled with noncaseating granulomas in many tissues and organs

A

Sarcoidosis

87
Q

Whos is sarcoidosis most common in?

A
  • female
  • blacks (10x more frequent)
  • rare in asian populations
88
Q

What are the three likely factors that cause sarcoidosis?

A
  • disordered immune regulation
  • genetic predisposition
  • environmental exposure
89
Q

What causes disordered immune regulation in sarcoidosis?

A

cell mediated (CD4+ helper T-cell) response to an unidentified antigen

90
Q

What could possibly be an environmental exposure cause of sarcoidosis?

A

possibly a microbe (Rikettsia, Propionibacterium, mycobacteria)

91
Q

(blank) typically affects the lung with non-caseating granulomas. The granulomas surround the airways and a transbronchial broncoscopy usually can make the diagnosis.

A

Sarcoidosis

92
Q

Usually sarcoidosis does not progress in the lung, but sometimes it does. And when that happens it results in (blank). We call this a (blank) lung. The granulomas, which are present in the interstitial space, result in (blank)

A

diffuse fibrosis.
honeycomb
fibrosis

93
Q

Sarcoidosis frequently affects the (blank) and (Blank) lymph nodes.

A

hilar and peribronchial

so you will often see bilateral hilar adenopathy

94
Q

How does sarcoidosis typically present?

A
  • Pulmonary manifestations
  • constitutional symptoms
  • Elevated ACE levels
  • Hypercalcemia
95
Q

What are the pulmonary manifestations of sarcoidosis?

A

SOB, cough, chest pain, hemoptysis

96
Q

What are the constitutional symptoms fo sarcoidosis?

A

Fever, fatigue, weight loss, anorexia, night sweats

97
Q

About 10% of sarcoid patients show (blank). Why does this happen?

A

hypercalcemia

results from increased 1,25 dihydroxyvitamin D from the granulomas of sarcoid. This increased Vit D levels causing increased intestinal absorption of calcium resulting in hypercalcemia.

98
Q

What organs does sarcoidosis affect?

A

all organs!

99
Q

How does sarcoidosis affect the lungs?

Where does sarcoidosis affect the lymph nodes?

How does sarcoidosis affect the spleen?

Where does sarcoidosis affect the bone marrow?

How does Sarcoidosis affect the skin?

How does sarcoidosis affect the eye?

A
  • small 1-2 cm lesions
  • hilar and mediastinal lesions
  • enlarged in 20%, affected (histologically) in 75%
  • hands and feet
  • variably (nodules, rash)
  • iritis, irdocyclitis
100
Q

(blank) is a chronic disease of unknown cause characterized by the enlargement of lymph nodes in many parts of the body and the widespread appearance of granulomas derived from the reticuloendothelial system.

A

Sarcoidosis

101
Q

What is this:
immune mediated, mostly interstitial lung disorders caused by abnormal sensitivity/reactivity to an inhaled organic antigen

A

Hypersensitivity Pneumonitis

102
Q

How come hypersensitivity pneumonitis is different than asthma?

A

because unlike astham, this involves the alveoli!

103
Q

What are syndromes name by associated with hypersensitivity pneumonitis?

A

Farmer’s lung
Pigeon breeder’s lung
Humidifier/Air-conditioner lung

104
Q

Where do you see hypersensitivity pneumonitis?

A

centered around bronchioles,

105
Q

What are the components of interstitial pneumonitis?

A
  • lymphocytes
  • plasma cells
  • macrophages
106
Q

What wil hypersensitivity pneumonia result in?

A 
Interstitial pneumonitis
Noncaseating granulomas
Intestitial fibrosis
Honeycombing
Obliterative bronchiolitis (late)
107
Q

What is pneumonia?

What is pneumonitis?

A

inflammation of alveoli

inflammation of the interstitium

108
Q

What are the clinic features of acute hypersensitivity pneumonitis?

A

Fever
dyspnea
cough
leukocytosis

109
Q

When does leukocytosis occur in acute hypersensitivity pneumonitis?

A

4-6 hrs after exposure

110
Q

How does chronic hypersensitivity pneumonitis present?

A

respiratory failure
dyspnea
cyanosis

111
Q

How can you prevent the progression from acute hypersensitivity pneumonitis to chronic pneumonitis?

A

early recognition and removal of causative agent can prevent progression

112
Q

What is pulmonary eosinophilia?

A

infiltration of eosinophils in the lungs

113
Q

What is the presentation of acute eosinophilic pneumonia with respiratory failure and what is it caused by?

A

unknown cause

-rapid onset of fever, dyspnea, hypoxemic respiratory failure.

114
Q

What is the clinical presentation of simple pulmonary eosinophilia? Is the course malignant or benign?

A
  • transient pulmonary lesions

- blood eosinophilia, benign clinical course

115
Q

What are the three type of pulmonary eosinophilias?

A
  • Acute eosinophilic pneumonia
  • Simple pulmonary eosinophilia
  • Chronic eosinophilic pneumonia
  • Secondary Eosinophilia
116
Q

What is the clinical presentation of chronic eosinophilic pneumonia?

A
  • focal areas of consolidation w/ lymps and eos
  • fever, night sweats, dyspnea
  • responds well to steroids
117
Q

What is the cause of secondary eosinophilia?

A

Usually due to infection, drug rxn, asthma, vasculitits, aspergillosis

118
Q

What is this:

accumulation of acellular surfactant in the intraalveolar and bronchiolar spaces

A

Pulmonary Alveolar Proteinosis

119
Q

What are the three types of pulmonary alveolar proteinosis?

A

Acquired
Congenital
Secondary

120
Q

What is the disease progression of congenital pulmonary alveolar proteinosis?

A

FATAL
typically noted in the newborn that develops rapidly progressive respiratory distress. Death within 3-6 mo w/o transplant

121
Q

Is secondary pulmonary alveolar proteinosis common?

A

uncommon, due to malignancies, immunodeficiencies, silicosis ..etc.

122
Q

What is the most common type of pulmonary alveolar proteinosis? What is it do to?

A

acquired (90% of all cases)

Likely due to antibody to granulocyte-macrophage colony stimulating factor (GM-CSF)
Autoimmune disorder

123
Q

How do you treat pulmonary alveolar proteinosis?

A

GM-CSF: about 50%
Whole lung lavage is standard of care

(GM-CSF: Granulocyte-macrophage colony-stimulating factor)

124
Q

What is pulmonary alveolar proteinosis similiar to?

A

acute silcosis

Block 9 Week 3 (6 decks)

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