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RESP 2304 Patho > Restrictive Disorders > Flashcards

Restrictive Disorders Flashcards

1
Q

What is Idiopathic Pulmonary Fibrosis?

A

It is a type of interstitial lung disease for which the cause is unknown. It is a progressive, chronic, and fatal condition characterized by excessive fibrotic tissue in the interstitial tissues of the lungs.

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2
Q

What are some other names for Idiopathic Pulmonary Fibrosis?

A

Usual Interstitial Pneumonia, cryptogenic fibrosing alveolitis, interstitial fibrosis.

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3
Q

What are some factors that may increase the risk of developing idiopathic pulmonary fibrosis?

A

Genetics, environmental exposures to dusts and tobacco, iatrogenic causes- medication or radiation, comorbidities such as GERD, obesity, emphysema.

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4
Q

What role does inflammation play in idiopathic pulmonary fibrosis?

A

It doesn’t. It may be present but it is not the primary driving factor.

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5
Q

What is the primary driving factor of idiopathic pulmonary fibrosis?

A

Aberrant response of the epithelial cells to micro-injuries. Fibro-genetic cytokines are released in response to injury which results in fibroblast proliferation. Also decreased responsiveness to apoptosis so normal pruning of tissue is limited and scar tissue develops uninhibited.

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6
Q

What is TGF-B?

A

Transforming growth factor B1 is a secrete protein that performs many cellular functions, including control of cell growth, proliferation, differentiation, and apoptosis. It enhances the remodeling of lung interstitium in idiopathic pulmonary fibrosis.

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7
Q

What happens to Type I and Type II pneumocytes in idiopathic pulmonary fibrosis?

A

The integrity of the basement membrane as well as the Type I pneumocytes is lost, and rapid growth of Type II pneumocytes occurs to re-establish the barrier as there is a signal failure to stop proliferation.

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8
Q

Describe the disease process of idiopathic pulmonary fibrosis.

A
  1. Some initial injury damages alveolar surface and basement membrane, we have formation of a wound clot and initiation of platelets and fibrin
  2. We re-establish the extracellular matrix, new vessels form, alveolar type II cells re-establish barrier
  3. There is a lack of pruning of the new extracellular matrix and lack of conversion of AECII cells to AECI cells
  4. The tension provided by the extracellular matrix on the airways is what produces the honeycomb pattern on HRCT which eventually causes traction bronchiectasis
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9
Q

What are the signs and symptoms of idiopathic pulmonary fibrosis?

A

Dry and non-productive cough, new onset exertional dyspnea, finger clubbing, fine late inspiratory crackles

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10
Q

What is an important thing to ask about in the patient’s history when you suspect idiopathic pulmonary fibrosis?

A

A detailed occupational history and any potential exposures to causative agents (organic/inorganic dust, tobacco smoke, etc.)

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11
Q

What might you expect to see on inspection and on an ABG of someone with idiopathic pulmonary fibrosis?

A

High RR and shallow Vt; Unless the patient is very end-stage they often have respiratory alkalosis

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12
Q

What receptors are stimulated and cause shallow breathing in idiopathic pulmonary fibrosis patients?

A

Pulmonary irritant receptors (increased traction due to restriction) and Juxtacapillary receptors (fibrotic changes to A/C membrane or interstitium.

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13
Q

Is the rapid shallow breathing in idiopathic pulmonary fibrosis due to chemical or non-chemical factors?

A

Non-chemical

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14
Q

What disease may pulmonary fibrosis lead to if left untreated?

A

Pulmonary hypertension; could see JVD and peripheral edema

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15
Q

What findings may you observe on CXR of someone who has pulmonary fibrosis?

A

Bilateral lower lobe opacities and possible decrease in lung volumes

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16
Q

What lab findings may be found in a patient with pulmonary fibrosis?

A

Results of blood tests are generally normal and there is no associated systemic disease because it is isolated to the lungs.

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17
Q

How do we diagnose idiopathic pulmonary fibrosis?

A

It requires exclusion of known causes like environmental exposure, medications, and systemic diseases

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18
Q

What diagnostic tools would we use to diagnose idiopathic pulmonary fibrosis?

A

CT, lung biopsy, PFT indicating restrictive disease, BAL to rule out other inflammatory causes.

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19
Q

What are the goals of therapy for a patient with idiopathic pulmonary fibrosis?

A

To slow progression, improve function, comfort, and avoid complications (we cannot cure or reverse the disease)

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20
Q

What is Pirfenidone?

A

Used to treat IPF. Anti-inflammatory, antioxidant, antifibrotic; used to slow progression

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21
Q

What is Nintedanib?

A

Use to treat IPF. Inhibits fibroblast, vascular, and platelet derived growth factors and slows decline in lung function.

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22
Q

What drug may be used to treat IPF but is still under investigation?

A

Thalidomide. It is anti-inflammatory, anti-angiogenic, and immunomodulatory; suppressed fibrotic response in animal models.

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23
Q

When IPF has progressed enough to cause pulmonary HTN, what is a treatment we may use?

A

Sildenafil: a phosphodiesterase 5 inhibitor that dilates well ventilated vessels.

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24
Q

What are some alternative therapies for pulmonary fibrosis?

A

Lung transplantation, pulmonary rehab, prevention of exacerbation

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25
Q

Why should we avoid invasive ventilation in pulmonary fibrosis patients?

A

High RR can lead to poor synchrony and there is a high mortality (87%) once intubated.

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26
Q

What is pleural effusion?

A

An accumulation of an abnormal amount of fluid in the pleural cavity.

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27
Q

What are the two types of effusion?

A

Transudative and exudative.

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28
Q

What is transudative effusion?

A

Fluid accumulating as a result of disturbance of the balance between transcapillary pressure and plasma oncotic pressure. Fluid moves from pulmonary capillaries into the pleural space. It is thin, watery, and has low protein and blood cell counts.

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29
Q

What is exudative effusion?

A

Fluid formation due to increased capillary permeability. It has a higher protein and cell count. Generally caused by inflammation, infection, or malignancy.

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30
Q

What is neoplasm?

A

A disturbance of the normal reabsorption of fluid secondary to the obstruction of the mediastinal lymph nodes draining the parietal pleura

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31
Q

What fluids can get into the pleural space (other than transudate and exudate)?

A

Pus (empyema), blood (hemothorax), chyle (chylothorax).

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32
Q

What are the signs and symptoms of a pleural effusion?

A

Chest pain, dyspnea, increased RR, decreased fremitus, mediastinal shift to opposite sides in severe cases, dry, non-productive cough, JVD

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33
Q

What is the key diagnostic tool for diagnosis of pleural effusions?

A

CXR showing density over the affected area; entire side of lung will be obscure and might have meniscus sign.

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34
Q

What does the pH value of a thoracentesis tell us about a pleural effusion?

A

<7.2- empyema
7.2-7.3 - take in clinical context; watch and wait
>7.4- CHF/malignancy
7.6 is normal

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35
Q

What is the safe triangle?

A

The safe place to put a chest tube. Usually in the 4th or 5th intercostal space in the axillary zone. We want to put it as high as possible so it does not damage the diaphragm

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36
Q

What is the best treatment for a pleural effusion?

A

To treat whatever else that is going on to cause it. (ex. heart failure, lung infection, etc.)

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37
Q

What is pleurodesis?

A

Injecting a substance such as tetracycline (antibiotic) directly into the pleural cavity to cause the surface of the lung to adhere directly to the chest wall, reducing recurrent effusions.

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38
Q

What is PleurX?

A

An indwelling pleural catheter used to treat recurring pleural effusions.

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39
Q

What is a pneumothorax?

A

Air within the pleural space

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40
Q

What are the three types of pneumothorax?

A

Closed, open, and tension

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41
Q

What is a closed pneumothorax?

A

Air enters the pleural space from the lung through a tear in the visceral pleura

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42
Q

What is an open pneumothorax?

A

A puncture through the chest wall that allows air in from the outside.

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43
Q

What is pendelluft?

A

A phenomenon created by an open pneumothorax where air from the collapsed lung is pushed into the unaffected lung and the mediastinum swings towards unaffected side, compressing it. On exhalation, air escapes through the wound and the mediastinum swings back towards collapsed lung. Because of this swinging, air is being re-breathed between the two lungs.

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44
Q

What is a tension pneumothorax?

A

A pneumothorax where air can enter the pleural space but cannot leave, so the chest is continuously filling with air. It compresses lungs and compromises cardiopulmonary function, so it is life-threatening.

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45
Q

What is the biggest sign of a tension pneumothorax?

A

Tracheal deviation and hyperinflation of chest.

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46
Q

Why do we place chest tubes above the ribs?

A

To avoid the nerve bundles and blood vessels that run in the grooves on the underside of the ribs.

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47
Q

What do we never do to chest tubes?

A

Clamp them

48
Q

What is hypersensitivity pneumonitis?

A

A hypersensitivity reaction affecting the lung parenchyma that occurs in response to inhaled organic dusts. Type III or IV reaction.

49
Q

What is a Type III allergic reaction mediated by?

A

IgG and IgM

50
Q

What is a Type IV allergic reaction mediated by?

A

T-cells

51
Q

How does hypersensitivity pneumonitis occur?

A

Inhalation of antigens, dust, or other organic material that causes thickening of alveolar walls and infiltration with lymphocytes, plasma cells, and eosinophils. This causes formation of granulomas and fibrotic changes in the lungs.

52
Q

How does hypersensitivity pneumonitis present?

A

Can be either acute or chronic. Farmers lung tends to be acute and avian worker lung tends to be chronic. Frequent acute episodes may develop into chronic presentation.

53
Q

What are the signs and symptoms of hypersensitivity pneumonitis?

A

Dyspnea, productive cough, clubbing, weight loss, fatigue. On CXR FIBROSIS OF UPPER LOBES, restrictive pattern

54
Q

What lab findings might you have in hypersensitivity pneumonitis

A

Abnormal serum proteins and presence of certain antibodies, presence of CD8+ lymphocytes in a BAL.

55
Q

What are some treatments for hypersensitivity pneumonitis?

A

Sometimes doesn’t require treatment; corticosteroids and bronchodilators for symptomatic relief.

56
Q

What protective effect does smoking have?

A

Smoking is protective against hypersensitivity pneumonitis because it increases the threshold for macrophage activation and decreases lymphocyte proliferation, also impairs T-cell signalling

57
Q

What is sarcoidosis?

A

A chronic, systemic granulomatous disease that may involve any organ (more common in lungs). Common sites are lung, skin, eye, lymphatics, spleen, and liver.

58
Q

What is the most common presentation of sarcoidosis?

A

Non-necrotizing granulomas of the lungs.

59
Q

How is it thought that sarcoidosis occurs?

A

Exact cause unknown, but may be triggered by exposure to a microbial agent in someone who has genetic susceptibility to the disease.

60
Q

What is a major hallmark of sarcoidosis?

A

Increases in the 3 major immunoglobulins (IgM, IgG, and IgA)

61
Q

What are some pathological findings with sarcoidosis?

A

Variable fibrosis, honeycombing and emphysematous changes, numerous granulomas.

62
Q

What CT changes would you see in someone who has sarcoidosis?

A

Enlarged lymph nodes and granulomas. Granulomas tend to form along areas rich in lymphatic tissue.

63
Q

What is a Kveim test? (This question is on the test)

A

A test to diagnose sarcoidosis where a part of a spleen form a patient with known sarcoidosis is injected into the skin of a patient suspected to have the disease. If non-caseating granulomas are found 4-6 weeks later, the test is positive.

64
Q

What is the treatment for sarcoidosis?

A

There isn’t really one, most cases go away on their own and those that do receive treatment it is to prevent scarring and damage to the affected organ. This may involve immunosuppressants.

65
Q

What is pneumoconiosis?

A

Interstitial lung disease of known etiology caused by inhalation of inorganic dusts.

66
Q

What are some examples of inorganic dusts that cause pneumoconiosis?

A

Silica, asbestos, coal miner lung

67
Q

How does pneumoconiosis develop?

A

Inorganic particles are deposited in the airway, these particles are phagocytized by macrophages and cause release of inflammatory mediators. Lysosomes cannot digest the particles so the oxidative agents are released back into the cell causing damage to multiple cells.

68
Q

What is the difference between chronic silicosis, accelerated silicosis, and acute silicoproteinosis?

A

Chronic is associated with >10 years of low level exposure while accelerated silicosis may appear in 5; acute silicoproteinosis onset time is <5 years due to large doses of silica which causes respiratory failure and death within a few months.

69
Q

Describe simple silicosis vs. complicated silicosis?

A

In simple, small nodules are scattered throughout the lungs and are small. In complicated, nodules join and form large masses of fibrous tissue, generally in the upper lobes and hilar region.

70
Q

What PFT pattern might we find in a patient with silicosis?

A

Any and all, so we cannot use PFT as a diagnostic tool on its own for pneumoconiosis.

71
Q

Why is asbestos so dangerous?

A

Once the fibers are in the body they never dissolve and the body has extreme difficulty expelling them, causing pneumoconiosis.

72
Q

What might you see on a CXR and CT of someone with asbestosis?

A

Ground-glass appearance, especially in lower lobes. Plaque along chest walls will distinguish asbestosis from IPF.

73
Q

How does Coal Miner’s lung develop?

A

Alveolar macrophages engulf the dust, release cytokines that stimulate inflammation, and collect in lung interstitium around bronchioles and alveoli (called coal macules). Coal nodules develop as collagen accumulates and focal emphysema develops as bronchiole walls weaken and dilate.

74
Q

What is anthracosis?

A

Pneumoconiosis caused by the accumulation of carbon in the lungs due to repeated exposure to air pollution or inhalation of smoke or coal dust particles.

75
Q

What test should be performed on patients with silicosis?

A

A manitoux (TB) test because the damage caused by silica increases the risk of the patient contracting TB.

76
Q

How do chemotherapy drugs affect the lungs?

A

Many disrupt the normal epithelium and break down natural barriers at the same time as weakening the immune system.

77
Q

What is Bleomycin and what does it do?

A

A chemo drug that causes oxidative damage to DNA and concentrates in skin and lungs.

78
Q

How does Bleomycin damage the lungs?

A

Causes type I pneumocyte destruction and type II pneumocyte hyperplasia leading to activation of fibroblasts, collagen deposition, and fibrosis.

79
Q

What are some risk factors associated with bleomycin?

A

O2 is a synergistic toxin so patients are sensitive to O2 therapy for up to 6 months, radiation increases risk of toxicity, and decreased renal function (especially associated with age >40) can increase risk of toxicity

80
Q

What are some symptoms of bleomycin toxicity?

A

Dyspnea, non-productive cough, low grade fever, decreased lung volume and elevated diaphragm

81
Q

How do you treat bleomycin toxicity?

A

Stop therapy, IV corticosteroids, supportive

82
Q

What is cyclophosphamide and what does it do?

A

An immune suppressant used in cancer treatment. It causes depletion of Gutathione (an antioxidant), making the patient more susceptible to oxidative stress. Glutathione is important in tissue building and repair.

83
Q

What drug may you suspect of toxicity if you see pleural thickening on a CXR?

A

Cyclophosphamide

84
Q

What is the treatment for cyclophosphamide toxicity?

A

Stop therapy, steroids, supportive measures

85
Q

What is methotrexate and what does it do?

A

It is a chemotherapy drug (a folate antagonist). It is a folate antagonist and causes a deficiency of folic acid, inhibits cell division, and is an immunosuppressant

86
Q

What lab findings are associated with methotrexate toxicity?

A

Eosinophilia and lymphocytic alveolitis (bronchoscopy) similar to a hypersensitivity reaction.

87
Q

What is amiodarone and what does it do?

A

It is an anti-arrhythmic that disrupts phospholipid breakdown, causing cell injury.

88
Q

What are common mechanisms of injury when inhaling toxic gas?

A

Asphyxiation, cellular injury

89
Q

Describe coagulation necrosis?

A

Burns caused by acidic gases, the cellular structure is maintained but enzymes, proteins, etc. are denatured or destroyed.

90
Q

Describe liquefaction necrosis?

A

Burns caused by alkaline gases. The cellular structure is destroyed. These tend to be more penetrating and therefore greater in severity than acid burns.

91
Q

What are some upper airway treatments for inhalation of toxic gas?

A

Removal from source, nebulized epi, cold neb, secure airway, O2 therapy, and IV steroids.

92
Q

What is reactive airways dysfunction?

A

Reactive airways dysfunction syndrome is caused by inhalation injury and the symptoms mimic asthma but are unresponsive to asthma treatments.

93
Q

What lower airway effects can be seen due to inhalation of toxic gas?

A

Leaky A-C membrane and edema

94
Q

What is hydrogen sulfide?

A

A toxic gas formed from decaying matter. Likely exposure comes from petroleum drilling, agriculture, and sewage treatment.

95
Q

How does hydrogen sulfide cause respiratory arrest?

A

Irritant signals through the vagus nerve and disrupts mitochondrial metabolism, decreasing ATP production and leading to death

96
Q

How does chlorine gas cause harm?

A

Most damage is done through oxidation but it may also become hypochlorous and hydrochloric acid that causes chemical burns of the airway, as well as act as an asphyxiant

97
Q

How does nitrogen dioxide cause harm?

A

Moisture in the airways reacts with it to form nitric and nitrous acids which are corrosive to the respiratory tract.

98
Q

How does carbon monoxide kill you?

A

It binds more strongly to hemoglobin than oxygen does so you cannot move oxygen around your body.

99
Q

How is carbon monoxide poisoning treated?

A

Aggressive oxygen treatment and hyperbaric oxygen.

100
Q

What is ARDS?

A

Respiratory failure in adults or children that results from diffuse injury to the endothelium of the lung. Characterized by pulmonary edema with and abnormally high amount of protein in the edematous fluid.

101
Q

What are the common etiologies associated with ARDS?

A

Sepsis, aspiration, pneumonia, shock, trauma

102
Q

What must we rule out in order to diagnose ARDS?

A

Cardiogenic pulmonary edema. (left heart failure) causing transudative edema

103
Q

What causes pulmonary edema in ARDS?

A

It is non-cardiogenic and due to increased permeability of capillary and alveolar epithelium due to damage. Produces exudate

104
Q

What is the difference between endothelial and epithelial cells?

A

Endothelial cells line blood vessels, epithelial cells line organs.

105
Q

What are the three phases of ARDS?

A

Exudative, proliferative, and fibrotic

106
Q

Describe the exudative phase of ARDS.

A

24-72 hours after injury. Destruction of type I cells and endothelial swelling. Influx of protein rich edema into the airspaces, lung inflammation, and neutrophil accumulation.

107
Q

Describe the proliferative phase of ARDS.

A

From 4-10 days. Proliferation of alveolar type II cells and fibroblasts, mild interstitial fibrosis. Exudative material forms hyaline membrane.

108
Q

Describe the fibrotic phase of ARDS.

A

Greater than 8 days. Formation of fibroblasts and scarring, fibrosis.

109
Q

What is the typical pattern of ARDS?

A

Initial or acute illness
Apparent respiratory stability
Respiratory deterioration and insufficiency
Terminal stage

110
Q

What are some clinical findings associated with ARDS?

A

Decreased compliance, regionally hyperinflated areas, decreased oxygenation, increased resistance, varying areas changing Raw and Cl throughout the lung

111
Q

What are some lab findings associated with ARDS?

A

Normal or increased WBCs, increased lactate as tissues are not getting enough O2, increased BUN, alkalosis progressing to acidosis on ABG

112
Q

What might we see on CXR of a patient with ARDS?

A

Fluffy whiteout bilaterally

113
Q

Why are lower tidal volumes helpful in ARDS?

A

The disease process is not homogenous so some areas are very restricted and fibrotic while others will hyperinflate so we have to be able to balance volumes and pressures it takes to open sicker areas without damaging the healthy tissues.

114
Q

What is the Berlin Definition?

A

Berlin Definition is used to define ARDS. It states that it must occur within a week of a known clinical insult or new or worsening respiratory symptoms, have bilateral opacities not explained by effusions, collapse, or nodules, and the respiratory failure is not fully explained by cardiac failure or fluid overload.

115
Q

What ventilation strategies can we use to treat a patient with ARDS?

A

We can use oscillation (HFOV) or APRV and other lung-protective strategies like permissive hypercapnia and high PEEP depending on the patient.

116
Q

What drug is particularly helpful for ARDS?

A

Cisatracurium. It reduces mortality, end organ failure, and ICU stay. It is a neuromuscular blocking drug that reduces patient effort during the critical phase, increases chest wall compliance, and allows the clinician to get a handle on lung mechanics.

RESP 2304 Patho (8 decks)

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