9.5 Restrictive diseases Flashcards

1
Q

sarcoidosis in lung:

-histology. what’s characteristic?

A
  • noncaseating granulomas, made of epitheloid histiocytes
  • characteristic ‘asteroid bodies’ seen inside giant cells of granulomas
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1
Q

“pigeon breeder’s lung”

A

ex of Hypersensitivity pneumonitis.

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2
Q

beryliosis

  • how presents
  • what populations
  • increased risk for what complication
A
  • pneumoconiosis caused by inhalation of beryllium–aerospace industry, beryllium miners
  • noncaseating granulomas in lung, hilar lymph nodes, systemic organs (just like sarcoidosis!)
  • increase risk of lung cancer
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3
Q

Asbestosis

-what population

A

-construction workers, plumbers, shipyard workers

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3
Q

sarcoidosis:

-general mech, etiology

A
  • etiology unknown,
  • probably a T-cell response to unknown antigen
  • noncaseating granulomas in systemic organs
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5
Q

what 2 drugs are notorious for causing interstitial fibrosis as side effect

A

amiodarone

bleomycin

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5
Q

sarcoidosis in lung:

-clinical presentation (3)

A
  1. dyspnea, cough
  2. elevated serum ACE
  3. hypercalcemia (increased 1A OHlase)
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6
Q

sarcoidosis:

-what electrolyte concentration is altered in blood? why?

A
  • hypercalcemia
  • noncaseating granulomas: the epitheloid histiocytes have 1-alpha-OHlase activity to convert Vit D to active form.
  • you see this in berylliosis too
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6
Q

sarcoidosis:

what enzyme is increased in blood?

A

elevated serum ACE

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7
Q

what is the only pneumoconioses that increases risk of TB?

A

silicosis

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8
Q

Hypersensitivity pneumonitis

  • clinical presentation
  • complication
  • mech
A
  • fever, cough, dyspnea hours after exposure to inhaled organic antigen. Resolves with removal, but chronic leads to interstitial fibrosis
  • inhaled organic antigen causes granulomatous reaction. Eosinophils involved.
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9
Q

Berylliosis

-what electrolyte concentration is altered in blood? why?

A
  • hypercalcemia
  • noncaseating granulomas: the epitheloid histiocytes have 1-alpha-OHlase activity to convert Vit D to active form.
  • you see this in sarcoidosis too
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10
Q

Coal miner pt with lung problems:

-what other problems to look for

A
  • Rheumatoid arthritis
  • (Caplan syndrome–“rheumatoid pneumoconiosis”)
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11
Q

idiopathic pulmonary fibrosis

  • progression of disease
  • tx
A
  • initially fibrosis seen on CT in subpleural patches
  • then, results in diffuse fibrosis with end-stage ‘honeycomb’ lung
  • tx: lung transplant
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11
Q

pneumoconioses

  • what is this
  • mech of disease
A
  • interstitial disease caused by long-term inhalation of small particles in occupational exposure
  • small particles are not removed by mucus and enter alveoli, where macrophages eat them. Macrophages don’t know what to do with these foreign particles and induce fibrosis of lung.
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12
Q

Fibrosis of lung, pleura,

cancer of lung, pleura.

-what pneumoconiosis?

A

Asbestosis

14
Q

Pt has noncaseating granulomas in lung, hilar lymph nodes, systemic organs, and works in aerospace industry

-suspect what?

A
  • Beryliosis!
  • sounds just like sarcoidosis
15
Q

anthracosis

A

collections of macrophages filled with carbon from inhalation. Very common in hilar lymph nodes, but mostly asymptomatic. Can be found in people that live in polluted cities.

17
Q

Asbestosis

  • complications, which is more common
  • how to diagnose?
A

-“fibrosis of lung, pleura,

cancer of lung, pleura”

  • lung cancer much more common than mesothelioma (pleura cancer)
  • dx with histology: asbestos bodies in lung (long fibers)
18
Q

sarcoidosis:

-how does it affect lung

A
  • noncaseating granulomas in lung and hilar lymph nodes
  • restrictive lung disease
20
Q

idiopathic pulmonary fibrosis

-what is considered most likely mech?

A

-cyclical lung injury induces TGF-beta healing response from injured pneumocytes. This induces fibrosis.

21
Q

what is the syndrome assoc with Coal worker’s pneumoconiosis?

A

Caplan syndrome:

combo of:

  1. diffuse interstitial fibrosis (‘black lung’)
  2. rhematoid arthritis
22
Q

idiopathic pulmonary fibrosis

-make sure to rule out what things?

A
  1. drugs: (bleomycin, amiodarone)
  2. radiation therapy
23
Q

sarcoidosis:

  • common clinical presentations (4)
  • progression of disease, tx
A

noncaseating granulomas throughout systemic organs. Common ones:

  1. lung fibrosis
  2. uveitis
  3. skin (cutaneous nodules, erythema nodosum)
  4. salivary, lacrimal glands (mimics Sjogrens)
    - Tx with steroids. often resolves spontaneously w/o tx
24
Q

restrictive lung disease:

2 major categories of mechanism

A

restricted filling of lung:

  1. interstitial disease
  2. chest wall abnormalities (eg obesity), so lungs cannot fill
25
Q

Pt with lung granulomas and eosinophils

-what is this

A

Hypersensitivity pneumonitis

-granulomatous reaction to inhaled organic antigen (eg pigeon breeder’s lung)

27
Q

Name the pneumoconioses:

A
  1. Coal workers’ pneumonconiosis
  2. silicosis
  3. berylliosis
  4. asbestosis
28
Q

silicosis

  • mech
  • how presents
A
  • silica impairs phagolysosome formation in macrophages. only pneumoconiosis that increases risk of TB infection
  • fibrotic nodules in upper lobes of lung
29
Q

sarcoidosis:

-classic population

A

black females