16 - Interstitial Occupational & Environmental Lung Disease Flashcards

1
Q

What is the general pathogenesis of interstitial lung disease?

A
  • injury or antigen exposure to alveolar epithelium/capillary –> acute inflammation (alveolitis) –> chronic inflammation (granuloma) and fibrosis
  • fibrosis can occur without inflammation and indicates an abnormal repair process after injury
  • disease can occur from single or repeated injury
  • some diseases are antibody related
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2
Q

Interstitial lung disease has [increased/decreased] compliance and [increased/decreased] elastic work of breathing.

A

Decreased compliance, increased elastic WOB

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3
Q

Why is hypercapnia not seen in someone with interstitial lung disease?

A

Because CO2 is more diffusible

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4
Q

What is the major cause of hypoxemia at rest in someone with interstitial lung disease? With exercise?

A

V/Q mismatch at rest, diffusion abnormality with exercise

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5
Q

Interstitial lung disease has [normal/increased] dead space.

A

Increased dead space, especially with rapid shallow breathing

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6
Q

What PFT patterns will be seen for someone with interstitial lung disease?

A
  • restrictive pattern - decreased FEV1, FVC, TLC, RV
  • decreased DLCO
  • normal or increased FEV1/FVC
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7
Q

What are the complications of pulmonary fibrosis?

A
  • respiratory failure
  • pulmonary emboli
  • lung cancer
  • pneumothorax
  • pulmonary hypertension
  • cor pulmonale
  • susceptibility to pulmonary infection
  • acute exacerbations
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8
Q

What are the clinical findings for interstitial lung disease?

A
  • progressive dyspnea
  • non-productive cough
  • rapid, shallow breathing
  • velcro inspiratory crackles
  • signs of cor pulmonale
  • clubbing may occur
  • multi-organ involvement may occur
  • lower extremity edema
  • tachypnea, tachycardia
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9
Q

What are the radiological findings that may be seen with interstitial lung disease?

A
  • bilateral diffuse infiltrates (reticular, nodular, or reticulonodular)
  • ground glass
  • honeycombing
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10
Q

What do reticular bilateral diffuse infiltrates on chest x ray suggest?

A

fibrosis (interstitial lung disease)

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11
Q

What do nodular bilateral diffuse infiltrates on chest x ray suggest?

A

granulomas or infection (interstitial lung disease)

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12
Q

What do reticulonodular bilateral diffuse infiltrates on chest x ray suggest?

A

tumor (interstitial lung disease)

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13
Q

In general, what does a ground glass appearance on chest x ray suggest?

A

inflammation –> acute onset

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14
Q

In general, what does a honeycombing appearance on chest x ray suggest?

A

fibrosis

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15
Q

What does IPF stand for?

A

idiopathic pulmonary fibrosis

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16
Q

Pulmonary fibrosis can take [weeks/months or years] to develop.

A

months or years

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17
Q

What are some common pulmonary disease that result in end stage pulmonary fibrosis?

A
  • hypersensitivity pneumonitis
  • sarcoidosis
  • asbestosis
  • connective-tissue disease
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18
Q

Idiopathic pulmonary fibrosis (IPF) is more common in [men/women].

A

men

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19
Q

What are the clinical findings associated with idiopathic pulmonary fibrosis (IPF)?

A
  • dyspnea
  • cough
  • velcro rales
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20
Q

DIP [is/is not] related to smoking. If related, does smoking cessation help?

A

Is related; yes, smoking cessation may be the best treatment

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21
Q

RB-ILD [is/is not] related to smoking. If related, does smoking cessation help?

A

Is related; yes, smoking cessation may be the best treatment

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22
Q

Langerhans Cell Histiocytosis [is/is not] related to smoking. If related, does smoking cessation help?

A

Is related; yes, smoking cessation may be the best treatment

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23
Q

IPF [is/is not] related to smoking. If related, does smoking cessation help?

A

Is related; no, there is no definite benefit from smoking cessation

24
Q

Which interstitial lung diseases affect other organs?

A
  • sarcoidosis

- connective tissue disease

25
Q

What is the pathogenesis of sarcoidosis?

A

immunologic response to an unknown antigen –> CD4 alveolitis –> well formed, non-caseating granulomas without necrosis

26
Q

What demographics more commonly have sarcoidosis?

A
  • younger ages
  • African Americans
  • Ireland
  • Iceland
  • Scandinavia
27
Q

Enlarged hilar and mediastinal notes +/- interstial infiltrates and well formed, non-caseating granulomas without necrosis suggest ___.

A

sarcoidosis

28
Q

What organs are affected by sarcoidosis?

A
  • lungs
  • eyes
  • brain
  • heart
  • liver
  • skin
29
Q

What enzyme is elevated in most sarcoidosis patients?

A

ACE (angiotensin converting enzyme)

30
Q

How is sarcoidosis treated?

A

treat with steroids; may remit spontaneously

31
Q

What are some medications that cause medication-related interstitial lung disease?

A
  • amiodarone (anti-arrhythmic)
  • bleomycin (chemo)
  • cyclophosphamide (chemo)
  • nitrofurantoin (antibiotic)
  • monoclonal antibodies
32
Q

What is the work up for someone with interstitial lung disease?

A
  • occupational history
  • review of systems looking for rheumatology overlap
  • medications
  • radiation exposure
  • high resolution CT
  • PFTs
  • ABGs
  • screenings for connective tissue disease
  • often need a lung biopsy
33
Q

What is the major mechanism that leads to occupational/environmental lung disease?

A

ineffective clearance (size of inhaled particle determines site of disease, immunological reaction determines nature of lung disease that results)

34
Q

What are the four major categories of occupational/environmental lung disease?

A
  • pneumoconiosis - fibrotic reaction to inhaled inorganic dust, often with ineffective clearance
  • hypersensitivity pneumonitis - exaggerated immunologic reaction, usually to inhaled organic dust
  • direct tissue injury (non-immunologic)
  • occupational asthma (most common form of occupational lung disease in the US)
35
Q

What occupations are at high risk for asbestos exposure?

A
  • pipe fitters
  • auto mechanics
  • construction workers
36
Q

What is the definition of asbestosis?

A

Exposure to asbestos + ILD/pulmonary fibrosis

37
Q

What can result from asbestos exposure?

A
  • asbestosis
  • pleural plaques
  • effusions
  • mesothelioma
38
Q

What occupations are at high risk for berylliosis?

A
  • dental technicians

- computer and aerospace industry

39
Q

Alveolar macrophages release cytokines that lead to fibroblast proliferation in which diseases?

A
  • asbestosis
  • silicosis
  • coal workers pneumoconiosis
40
Q

What are the major types of hypersensitivity pneumonitis?

A
  • bird fancier
  • humidifier
  • farmer’s lung
  • hot tub lung
41
Q

What immune cell is predominant in hypersensitivity pneumonitis?

A

lymphocyte (CD8+)

42
Q

What is the treatment for hypersensitivity pneumonitis?

A
  • remove from exposure

- steroids

43
Q

What is direct tissue injury?

A

an acute, severe disease manifested as acute lung injury and non-carcinogenic pulmonary edema (injury to alveolar-capillary membrane)

44
Q

What occupations are at risk for direct tissue injury? What is the dangerous inhalant in each case?

A
  • silo fillers (NO2)
  • metal workers/welders (galvanized metals)
  • cotton workers (endotoxin in unprocessed cotton)
45
Q

What are the symptoms of direct tissue injury?

A
  • cough
  • fever
  • flu-like
46
Q

How do you differentiate between occupational asthma and work-exacerbated asthma?

A

occupational asthma - asthma with onset after start of exposure (usually takes a few weeks)

work-exacerbated asthma - symptoms start very soon after the exposure begins

47
Q

What is reactive airways dysfunction syndrome (RADS)?

A

Irritant-induced asthma with airway hyperreactivity that occurs in response to a fume-induced injury to airways

48
Q

How do you treat reactive airways dysfunction syndrome?

A

steroids

49
Q

What does IIP stand for?

A

idiopathic interstitial pneumonia

50
Q

What does DPLD stand for?

A

diffuse parenchymal lung disease

51
Q

What does CEP stand for?

A

chronic eosinophilic pneumonia

52
Q

What does PLCH stand for?

A

pulmonary Langerhans cell histiocytosis

53
Q

What does DIP stand for?

A

desquamative interstitial pneumonia

54
Q

What does RB-ILD stand for?

A

respiratory bronchiolitis interstitial lung disease

55
Q

What does COP stand for?

A

cryptogenic organizing pneumonia

56
Q

What does LIP stand for?

A

lymphocytic interstitial pneumonia