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Block 9 week 3 > Restrictive pulmonary disease - Parks and Baker > Flashcards

Restrictive pulmonary disease - Parks and Baker Flashcards

1
Q

Restrictive lung diseases are characterized by…?

A

Reduced expansion of lung parenchyma and reduced total lung capacity.

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2
Q

Restrictive lung diseases of present with….?

A
  1. dyspnea
  2. tachypnea
  3. end-inspiratory crackles
  4. cyanosis
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3
Q

What changes in spirometry would you expect in restrictive lung disease?

A
  1. decreased TLC
  2. decreased DLCO
  3. decreased FVC
  4. normal to increased FEV1
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4
Q

Name the types of lung parenchymal and chronic interstitial and infiltrative diseases.

A
  1. fibrosing
  2. granulomatous
  3. eosinophilic
  4. smoking related
  5. other
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5
Q

Name the fibrosing diseases.

A
  1. idiopathic pulmnary fibrosis
  2. nonspecific interstitial pneumonia
  3. cryptogenic organizing pneumonia aka - BOOP
  4. lung disease from connective tissue disorders
  5. pneumoconiosis
  6. drug reactions
  7. radiation pneumonitis
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6
Q

Chronic progressing fibrosis leads to…?

A

Honeycomb lung pathology.

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7
Q

How is idiopathic pulmonary fibrosis diagnosed/identified?

A

It is a clinic-pathologic syndrome. It is identified by a characteristic clinical picture, X-ray changes and pathologic changes.

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8
Q

What type of pathological changes are seen in idiopathic pulmonary fibrosis?

A

The pattern of changes is called usual interstitial pneumonia or UIP. It includes sub-pleural fibrosis and temporal heterogeneity. The earliest lesions are called ‘fibroblastic focus’. UIP changes can be seen in other diseases too.

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9
Q

UIP is a pattern of pathological changes seen in what other types of diseases?

A
  1. connective tissue diseases
  2. chronic hypersensitivity pneumonia
  3. asbestosis
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10
Q

Is idiopathic pulmonary fibrosis a fatal disease?

A

Yes. It is often fatal in 3-5 years.

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11
Q

What is temporal heterogeneity?

A

A pattern of normal areas alternating with abnormal areas of varying stages.

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12
Q

Describe the new hypothesis for the pathogenesis of idiopathic pulmonary fibrosis.

A
  1. environmental factors (smoking, exposure to particles and fumes), age (greater than 50) and predisposition (genetic factors affecting pneumocytes) all lead to persistent epithelial injury with inflammation contributing
  2. . there is aberrant wound healing that leads to interstitial fibrosis
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13
Q

What are some treatment options for idiopathic pulmonary fibrosis (IPF)?

A
  1. new drugs such as nintedinib
  2. lung transplant
  3. immunosupressants
  4. acute exacerbations can be treated with steroids
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14
Q

What is the clinical course of IPF?

A
  1. insidious onset with dry cough and dyspnea
  2. onset around age 40-70
  3. hypoxemia and clubbing appears late
  4. gradual deterioration, +/- exacerbations
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15
Q

What is a type of fibrosing lung disease of unknown etiology that fails to show diagnostic features of any of the other well-characterized interstitial lung diseases?

A

Nonspecific interstitial pneumonia.

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16
Q

Describe the two types of nonspecific interstitial pneumonia.

A
  1. cellular - presents with mild to moderate chronic interstitial inflammation that is uniform OR patchy and occurs at younger age with a better outcome
  2. fibrosing - presents with diffuse or patchy interstitial fibrosis, no temporal heterogeneity or honeycombing present, occurs in an older population and has a worse outcome
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17
Q

Can nonspecific interstitial pneumonia occur with IPF?

A

Yes. Prognosis is only as good as the worst lesion.

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18
Q

How does nonspecific interstitial pneumonia present?

A

Presents with dyspnea and cough for several months.Onset is usually around 46-55 years of age.

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19
Q

What is cryptogenic organizing pneumonia?

A

An interstitial lung disease associated with polypoid plugs of loose organizing connective tissue - called Masson bodies. These are basically granulation tissue.

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20
Q

What is the morphology of cryptogenic organizing pneumonia?

A
  1. organizing refers to long term changes of fibrinous exudates
  2. presents with polypoid plugs of connective tissue
  3. no temporal heterogeneity
  4. no interstitial fibrosis or honeycombing
  5. underlying lung architecture is normal
  6. is idiopathic
  7. presents with patchy opacities on X-ray
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21
Q

What are some clinical findings for cryptogenic organizing pneumonia?

A

Cough and dyspnea.

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22
Q

Organizing pneumonia is not always idiopathic. How else might it present?

A

As a secondary condition to:

  1. infections or inflammatory injury
  2. viral and bacterial pneumonia
  3. inhaled toxins
  4. drugs
  5. connective tissue disease
  6. GVHD (graft vs. host disease)
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23
Q

What are some connective tissue disorders in which there can be pulmonary involvement?

A
  1. RA - 30-40% of patients may get chronic pleuritis with or without effusion, diffuse interstitial pneumonitis and fibrosis, intrapulmonary rheumatoid nodules and pulmonary hypertension
  2. Systemic sclerosis - some patients may get diffuse interstitial fibrosis with UIP or non-specific interstitial pneumonia (more commonly)
  3. SLE - Some patients may present with patchy, transient parenchymal infiltrates and occasionally with severe lupus pneumonitis
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24
Q

Define pneumoconioses.

A

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

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25
Q

What are some major factors associated with the development of a pneumoconioses?

A
  1. particle size - 1-5 um particles are most dangerous since size allows them to settle in the distal airways
  2. amount of dust retained
  3. solubility and reactivity of the substance inhaled
  4. additive effects from other irritants such as smoking
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26
Q

Small particles are more likely to what…?

A

Cause acute lung injury.

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27
Q

Large particles are more likely to what…?

A

Settle in the lung parenchyma and evoke fibrosing collagenous pneumoconiosis.

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28
Q

Describe the pathophysiology of pneumoconiosis.

A
  1. dust, particles, chemicals are inhaled
  2. macrophages phagocytose the particles
  3. macrophages release mediators such as IL-1, TNF-a, ROS’s leading to continual cell injury
  4. fibroblasts proliferate and collagen is deposited in response to injury (interstitial cell fibrosis)
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29
Q

How does smoking contribute to pneumoconioses?

A
  1. smoking reduces mucociliary clearance and increases inflammation
  2. less clearance = more retained particulate matter
  3. worsens the effects of all inhaled dusts and is particularly magnified in the setting of asbestos inhalation
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30
Q

What are the 3 main types of inhalational exposures that we may see clinically?

A
  1. coal worker’s pneumoconiosis
  2. silicosis
  3. asbestos-related diseases
31
Q

What is the spectrum of disease that can be seen in coal worker’s pneumoconiosis?

A
  1. anthrocosis - presence of coal in the lungs, usually asymptomatic
  2. simple CWP
  3. complicated CWP
32
Q

Is there usually a decrease in lung function with anthrocosis and simple CWP?

A

No. There is usually minimal to no decrease in lung function.

33
Q

Is there a decrease in lung function with complicated CWP?

A

A minority of patients with complicated CWP develop progressive massive fibrosis. This is associated with emphysema and chronic bronchitis and decreased lung function.

34
Q

Is there an increased risk of cancer or TB with Coal worker’s pneumoconiosis?

A

No.

35
Q

Silicosis is a type of pneumoconiosis. What causes it?

A

Silicosis is caused by inhalation of dust - most often quartz. Pure quartz is worse than quartz mixed with other materials. This is seen in foundry workers, sandblasting, mining and stone cutting.

36
Q

What is the most prevalent occupational disease in the world?

A

Silicosis

37
Q

Describe the pathophysiology of silicosis.

A
  1. slowly progressive from decades of exposure
  2. causes nodular, fibrosing lesions
  3. tiny nodules form early on and then coalesce into hard collagenous scars
  4. nodules present usually in the upper zone
  5. usually there is no SOB until there is massive fibrosis
  6. possibly carcinogenic
38
Q

Describe acute silicosis.

A
  1. presents with abundant lipoproteinaceous material in the alveoli
  2. identical to alveolar proteinosis
39
Q

What might you see on chest X-ray with silicosis?

A
  1. upper zone nodularity

2. eggshell calcification in the hilar lymph nodes

40
Q

Does silicosis cause increased risk of TB infection?

A

Yes - it is possibly immune related.

41
Q

Asbestos exposure can lead to what in the pleura?

A
  1. pleural effusions
  2. fibrous plaques
  3. diffuse pleural fibrosis
  4. mesothelioma
42
Q

Asbestos exposure can lead to what in the lung parenchyma?

A
  1. lung carcinoma

2. interstitial fibrosis (asbestosis)

43
Q

What are the two forms of asbestos?

A
  1. serpentine/curly

2. amphibole/needle like

44
Q

Describe serpentine asbestos.

A
  1. most commonly used in industry

2. least likely to cause pathology because they tend to get stuck higher up in the lung

45
Q

Describe amphibole asbestos.

A
  1. less prevalent
  2. more likely to cause pathology because can penetrate deeper
  3. only form associated with mesothelioma
46
Q

How does asbestos lead to cancer?

A

Asbestos fibers adsorb toxic chemicals, increasing cancer potential.

47
Q

If you have been exposed to asbestos - does smoking increase your risk of lung carcinoma?

A

Yes. Asbestos exposure alone increases it 5X and if you smoke also then the risk increases 55X.

48
Q

If you have been exposed to asbestos - does smoking increase your risk of mesothelioma?

A

No. The asbestos exposure is the bigger risk factor for mesothelioma.

49
Q

Describe Asbestosis.

A
  1. causes diffuse pulmonary interstitial fibrosis
  2. will see ferruginous (asbestos) bodies - asbestos fiber coated with iron and containing proteinaceous material
  3. eventually causes honeycombing of the lungs
  4. changes similar to UIP
  5. lower lung and sub pleura areas are first affected
50
Q

What are the clinical features of asbestosis?

A
  1. early - dyspnea on exertion
  2. later - dyspnea at rest too
  3. productive cough
  4. rare to get sooner than 10 years after exposure, more often disease presents more than 20 years after exposure
51
Q

What effects does asbestos have on the pleura?

A
  1. plaques - well circumscribed, dense with collagen and calcium
  2. plaques more often in anterior and posterolateral parietal pleura, common over domes of diaphragm
  3. pleural effusions - usually serous
  4. rare pleural fibroses
  5. possible mesothelioma
52
Q

What types of granulomatous disease are associated with fibrosing pulmonary pathology?

A
  1. sarcoidosis

2. hypersensitivity pneumonitis

53
Q

Describe sarcoidosis.

A
  1. systemic disease of unknown cause
  2. causes noncaseating granulomas in many tissues and organs and can cause scarring and fibrosis
  3. affects females much more than males
  4. affects blacks 10X more frequently than whites
  5. rare in asian population
54
Q

What is the pathogenesis of sarcoidosis?

A

3 factors likely:

  1. disordered immune regulation - cell mediated, CD4 T-helper response to an unidentified antigen
  2. genetic predisposition - familial and racial clustering seen
  3. environmental exposure - possibly a microbe such as rikettsia, proprionibacterium or mycobacteria
55
Q

Granulomas can be caveating and non-caseating. In which conditions are you likely to see each?

A
  1. caseating granuloma - TB

2. non-caseating granuloma - sarcoidosis

56
Q

What type of lung pathology might you see with sarcoidosis?

A
  1. noncaseating granulomas
  2. honeycomb lung due to scarring and fibrosis
  3. on CXR a classic finding is enlarged hilar lymph nodes
57
Q

What clinical findings are associated with sarcoidosis?

A
  1. most present with pulmonary manifestations such as SOB, cough, chest pain, hemoptysis
  2. constitutional signs - fever, fatigue, weight loss, anorexia, night sweats
  3. elevated ACE levels
  4. hypercalcemia
58
Q

What organs does sarcoidosis effect?

A

Virtually no organ is spared:
Lungs – usually small, sometimes coalescing into 1-2cm lesions
Lymph Nodes – mostly hilar and mediastinal
Spleen – enlarged in 20%, affected (histologically) in 75%
Liver – affected less often than the spleen
Bone Marrow – hands and feet
Skin – variable (nodules, rash)
Eye – iritis, irdocyclitis
Salivary Glands
Muscle

59
Q

What is hypersensitivity pneumonitis?

A

An immune mediated, mostly interstitial lung disorder caused by abnormal sensitivity/reactivity to an inhaled organic antigen.

60
Q

How is hypersensitivity pneumonitis different from asthma?

A

HP involves the alveoli while asthma does not.

61
Q

Hypersensitivity pneumonitis is a general term. How is the condition more often specified?

A

Usually by association with what is the likely cause. For example:

  1. Farmer’s lung
  2. Pigeon breeder’s lung
  3. humidifier/AC lung
62
Q

Describe the pathophysiology of hypersensitivity pneumonitis.

A
  1. centered around bronchioles
  2. causes interstitial pneumonitis with lymphocytes, plasma cells and macrophages
  3. causes noncaseating granulomas
  4. causes interstitial fibrosis, honeycombing, and later may cause obliterative bronchiolitis
  5. early recognition and removal of the causative agent can prevent progression to serious chronic fibrotic disease
63
Q

What is the difference between pneumonia and pneumonitis?

A
  1. pneumonia= inflammation in the alveoli

2. pneumonitis - inflammation in the interstitium

64
Q

Describe the clinical features of acute hypersensitivity pneumonitis.

A

About 4-6 hours after exposure presents with fever, dyspnea, cough, and leukocytosis.

65
Q

Describe the clinical features of chronic hypersensitivity pneumonitis.

A

Continuous exposure results in respiratory failure, dyspnea and cyanosis.

66
Q

What is pulmonary eosinophilia?

A

Infiltration of eosinophils in the lungs.

67
Q

What are the types of pulmonary eosinophilia?

A
  1. acute eosinohilic pneumonia with respiratory failure - unknown cause, rapid onset of fever, dyspnea and hyperemic respiratory failure. 25% of eosinophils in BAL fluid, responds to steroids
  2. simple pulmonary eosinophilia - transient pulmonary lesions, blood eosinophilia, benign clinical course
  3. chronic eosinophilic pneumonia - focal areas of consolidation with lymphocytes and eosinophils, fever, night sweats, dyspnea, responds to steroids
  4. secondary eosinophilia - usually due to infection, drug rxn, asthma, vasculitis, aspergillosis
68
Q

What is pulmonary alveolar proteinosis?

A

Accumulation of acellular surfactant in the intra-alveolar and bronchiolar spaces.

69
Q

What are the three types of pulmonary alveolar proteinosis?

A
  1. acquired
  2. congenital
  3. secondary
70
Q

Describe acquired pulmonary alveolar proteinosis.

A
  1. 90% of all cases
  2. likely due to antibody to granulocyte-macrophage colony stimulating factor (GM-CFS)
  3. autoimmune disorder
71
Q

Describe congenital pulmonary alveolar proteinosis.

A
  1. fatal and typically noted in newborns that develop rapidly progressing respiratory distress
  2. survival is 3-6 months without transplant
72
Q

Describe secondary pulmonary alveolar proteinosis.

A
  1. uncommon

2. due to malignancy, immunodeficiency, silicosis etc.

73
Q

How is acquired pulmonary alveolar proteinosis treated?

A
  1. GM-CSF - about 50%

2. whole lung lavage is standard of care

74
Q

What is BAL?

A

bronchiole alveolar lavage

Block 9 week 3 (5 decks)

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