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KK RESPII Exam 1 > Gomez - Interstitial Diseases > Flashcards

Gomez - Interstitial Diseases Flashcards

1
Q

What type of appearance do infiltrative X-rays have?

A

Reticulonodular

Ground glass

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2
Q

What is another name for Idiopathic Pulmonary Fibrosis?

A

Usual interstitial pneumonia

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3
Q

Describe Idiopathic Pulmonary Fibrosis

A

Repeated bouts of inflammation lead to scarring of the lung

Cause of inflammation is largely unknown

Scarring causes increased thickness of diffusion barrier, inhibiting effectiveness of gas exchange

Very patchy

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4
Q

What does caveolin do?

Where is it found?

What is it inhibited by?

A

Inhibits deposition of collagen and ECM

Found in fibroblasts

TGF-β1

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5
Q

What are the typical sx of IPF?

A

Dyspnea

Cough

Hypoxemia with cyanosis

Clubbing of digits

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6
Q

What is the typical affected age group by IPF?

What is the median survival?

A

Typically affects middle-aged individuals

Median survival 3 yrs

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7
Q

What is the pathological progression of IPF?

A

Repeated bouts of inflammation causes scarring

Healing/scarring cycle causes patcy fibrosis

Over time, more and more areas of lung become involved

End result: honeycomb lung

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8
Q

What part of the lung does IPF affect first?

A

Usually affects sub-pleural region first, then progresses to involve more and more of lung

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9
Q

Describe Nonspecific Interstitial Pneumonia

A

Appears to be more limited than IPF

Not a result of repeated bouts of inflammation

Better prognosis than IPF

Will see inflammatory cells in interstitium

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10
Q

What is organizing pneumonia?

A

Replacement of tissue in the alveolar space by fibrous connective tissue

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11
Q

What are Masson bodies?

A

Balls of fibrous connective tissue that fill alveoli in cryptogenic organizing pneumonia

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12
Q

Describe cryptogenic organizing pneumonia

A

A pneumonia of unknown etiology in which fibrous connective tissue fills the alveolar spaces

No interstitial fibrosis, so no progression to honeycomb lung

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13
Q

How are systemic conditions related to lung diseases? What are some of them?

A

Autoimmune diseases can have lung involvement

Rheumatoid arthritis

Scleroderma

SLE

Sjögren synrome

Polymyositis/dermatomyositis

Mixed connective tissue disorder

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14
Q

What process is pictured here? What can it be a sign of?

A

Follicular bronchiolitis

Can indicate rheumatoid arthritis

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15
Q

What is Caplan syndrome?

A

Combination of rheumatoid arthritis and pneumoconiosis

Pneumoconiosis is disease caused by inhalation of something

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16
Q

What sized particles are most dangerous to lungs?

A

1-5 microns

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17
Q

What factors contribute to particulates entering lung tissue?

A

Amount/volume

Size, shape, and buoyancy of particles

Solubility/cytotoxicity

Concomitant irritants

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18
Q

Is a pt exposed to asbestos more likely to develop mesothelioma or carcinoma of the lung?

A

Carcinoma of the lung

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19
Q

What is Farmer’s lung?

A

Exposure to moldy hay

20
Q

What are the three stages of Coal Worker’s Pneumoconiosis (CWP)?

A

Asymptomatic anthracosis (coal dust inhalation)

Simple CWP

Complicated CWP

21
Q

What is anthracosis?

A

Deposition of black pigment in lung and adjacent lymph nodes

From coal dust

22
Q

Describe simple coal worker’s pneumoconiosis

A

Usually benign

Will see some macules and nodules that appear on X-ray

Pt has no difficulty breathing

23
Q

Describe complicated coal worker’s pneumoconiosis

A

Symptomatic form of CWP, often associated with progressive massive fibrosis (PMF)

Extensive scarring

Can result in pulmonary HTN and cor pulmonale

24
Q

What is depicted in this image?

A

Anthracosis

25
Q

What will make coal worker’s pneumonia significantly worse?

A

Concomitant exposure to silica

26
Q

Who is at greatest risk for developing silicosis?

A

Sandblasters

Miners

Demolition crews

27
Q

Describe the process of silicosis

A

Slowly progressive disease that takes decades to develop

Slica particle taken into lungs

Particle ingested by MΦ

MΦ killed by particle, causing it to be released along with inflammatory mediators

Particle can be taken up by other MΦ and process repeats

28
Q

What does silicosis look like on X-ray?

A

Egg shell calcification

29
Q

What conditions are related to asbestos exposure?

A

Pleural effusion

Pleural plaques or diffuse pleural fibrosis

Asbestosis

Lung CA

Laryngeal CA

Mesothelioma

30
Q

What are the two types of asbestos?

A

Chrysotile (more common in industry); wavy and larger particle, not as carcinogenic

Amphibole (more harmful form)

31
Q

What is shown in this image? Why is it brown?

A

Asbestos body

Brown because of iron content

32
Q

Do pleural plaques contain asbestos bodies?

A

No asbestos bodies present in pleural plaques

33
Q

What do pleural plaques look like on X-ray?

A

Pleural plaques appear as masses

Need to do multiple images or use another modality (CT or MR) to confirm presence

34
Q

What is Farmer’s lung caused by?

A

Moldy hay carrying Micropolysporia faeni

35
Q

What causes bagassosis?

A

Moldy pressed cane sugar colonized by thermophilic actinomycetes

36
Q

What causes humidfier lung?

A

Cool mist humidifiers colonized by thermophilic actinomycetes or Aureobasidium pullulans

37
Q

What causes bird fancier’s lung/pigeon breeder’s lung?

A

Serum proteins in pigeon droppings

38
Q

What is desquamative interstitial pneumonia?

A

Associated with smoking

A bunch of alveolar MΦs eating up all the junk that’s been inhaled

39
Q

What are Birbeck granules?

A

Found within Langerhans cells

Trilaminar in appearance with dilation at the end

Sometimes resemble tennis racquets, depending upon plane of section

40
Q

Describe Respiratory Bronchiolitis-associated Interstitial Lung DIsease (RBILD)

A

Commonly found in smokers

MΦ infiltrates in bronchioles

Can find peribronchiolar fibrosis

41
Q

What markers do Langerhans cells typically express?

A

CD1a, CD207 are the most common

Can also see S-100

42
Q

What are the characteristics of sarcoidosis?

A

Formation of numerous non-caseating granulomas

Can occur almost anywhere in the body

More common in women than men

More common in African Americans than Whites

43
Q

What testing can be abnormal in pts with sarcoidosis?

Why does this happen?

A

Pts can have anergy to PPD or Candida antigens

Due to immune system working so hard to address the sarcoidosis that it will not respond to other antigens

44
Q

What happens to angiotensin converting enzyme in sarcoidosis?

A

ACE level will be elevated in pts with sarcoidosis

Also elevated in pts with leprosy

45
Q

What is Löeffler’s syndrome?

A

Transient acute pulmonary eosinophilia from any cause

46
Q

What will be seen histologically in pts with pulmonary alveolar proteinosis?

A

Accumulation of acellular surfactant in the alveolar and bronchiolar spaces

47
Q

How would you treat autoimmune pulmonary alveolar proteinosis?

A

With granulocyte/macrophage colony stimulating factor (GM-CSF)

KK RESPII Exam 1 (9 decks)

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