Restrictive Lung Diseases and ILD Flashcards

1
Q
A
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2
Q

What is the definition of a restrictive lung disease?

A
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3
Q

What are iatrogenic causes of ILD?

A
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4
Q

What are the types of IPF?

A

usual interstitial pneumonia
nonspecific interstitial pneumonia
cryptogenic organizing pneumonia
acute interstitial pneumonia

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5
Q

What are the types of granulomatous ILD?

A

Sarcoidosis
hypersensitivity pneumonitis (organic dust)
farmer’s lung (actinomyces, hay, mold exposure)
Bird fancier’s lung - exposure to avian proteins
Tb

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6
Q

What CTDs cause lead to ILD?

A
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7
Q

List the types/ causes of ILD.

A
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8
Q

Other than ILD, what are some causes of restrictive lung diseases?

A
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9
Q

Classify all the causes of restrictive lung disease.

A
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10
Q

What gender is more likely to get the following restrictive lung diseases:
1. Sarcoidosis?
2. IPF?

A
  1. F>M
  2. M>F
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11
Q

What is the most common type of IPF (Give the full name)? What would you expect on histology and HRCT?

A
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12
Q

The “types of IPF” are really just the patterns observed on imaging and histology. What are all the findings on imaging of IPF (honours- give associated disease)

A

CXR - Ground-glass appearance (NIP - Non-specific interstitial pneumonia)
HRCT - Honeycombing +/- Bronchiectasis (=> Bronchial thickening, Increased Bronchoaterial ratio >1.5 (UIP), lack of tapering).
Histology: Honeycombing + Fibroblastic Foci

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13
Q

What are the RFs for ILD?

A
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14
Q

What is the most common presentation of sarcoidosis and what symptoms would the patient have?

A

Lofgren’s Syndrome

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15
Q

What symptoms would you like to elicit from a history of sarcoidosis?

A
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16
Q

Outline the questions you would ask in a history for ILD.

A
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17
Q

This is a patient with sarcoidosis. What does this image show?

A

Lacrimal gland enlargement in sarcoidosis

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18
Q

This is a patient with sarcoidosis. What does this image show?

A

Parotid enlargement in sarcoidosis

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19
Q

This is a patient with sarcoidosis. What does this image show?

A

cutaneous lesions (lupus pernio)

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20
Q

This is a patient with sarcoidosis.
What does this image show?

A

Erythema nodosum

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21
Q

This is a patient with sarcoidosis.
What does this image show?

A

anterior uveitis

22
Q

If there is one thing you would like to elicit during a clinical exam that would make you most suspicious of ILD, what is it? and what is the finding?

A

Auscultation of the lung bases
Bibasal fine end-inspiratory crackles

23
Q

The main signs of ILD that you cannot miss on exam are easily remembered via 3Cs. What are the 3 Cs?

A

Cough (dry cough)
Clubbing
Crackes (Fine bibasal end-inspiratory crackles)

24
Q

Outline the exam findings you would expect to find in ILD.

A
25
Q

What are the ddx for upper lobe fibrosis?

A
26
Q

What are the ddx for Lower lobe fibrosis?

A
27
Q

What are the ddx for Lung fibrosis?

A
28
Q

DLCO as part of PFTs can be affected by a few factors. What is the main one?

A

Anaemia => must be corrected as it affects the gradient (less Hb => less diffusion gradient)

29
Q

You are investigating a suspected ILD. Interpret the following results:
1. The DLCO comes back low.
2. The DLCO comes back normal.

  1. What is the DLCO and what does it assess?
A
30
Q

What are the diagnostic investigations for restrictive lung diseases?

A
31
Q

What would you expect on the HRCT of the following pathologies:
1. IPF
2. Sarcoidosis
3. Asbestos

A
32
Q

When is a surgical biopsy indicated in the setting of ILD? How is it done?

A

IF HRCT and Spirometry and not consistent & sarcoidosis
Bronchoscopy or EBUS

33
Q

In the setting of ILD, what cause of ILD necessitates ordering histology of the biopsy? What would you expect to see?

A

Sarcoidosis - non-caseating granuloma

34
Q

In a patient with ILD, what is the purpose of a 6 minute walk test?

A
35
Q

What is the main finding on ECHO you are looking for in severe ILD?

A

Severe ILD => severe fibrosis => increasing pulmonary pressure => Pulmonary HTN

=> on ECHO you will see cor pulmonale (dilated IVC, RA…) + estimated Pulmonary wedge pressure

36
Q

An ECHO can be used to estimate Pulmonary wedge pressure. How is this accurately measured?

A

Pulmonary Wedge Pressure is measured via a pulmonary artery catheter in the right side (@ the bifurcation)

37
Q

What investigations would you order for a patient presenting with symptoms indicative of restrictive lung diseases?

A
38
Q

Outline the preventative strategies for restrictive lung diseases.

A
39
Q

Outline the acute management of Restrictive lung diseases.

A

O2 and steroids (best for inflammatory cases –> all ILD EXCEPT FOR IPF.

40
Q

Outline the chronic management of IPF. Include SEs of the medications.

A
41
Q

Outline the chronic management of Sarcoidosis. Include SEs of the medications.

A
42
Q

What is the difference in etiology between hypersensitivity pneumonitis and pneumoconiosis?

A

Hypersensitivity pneumonitis from organic dust
Pneumoconiosis from inorganic dust

43
Q

Outline the acute and chronic management of Inhalation ILD.

A
44
Q

Outline the chronic management of chest wall disease

A

If needed, based on 6 minute walk test for portable O2

45
Q

Outline the full management of a patient with restrictive lung disease.

A
46
Q

What are the complications of restrictive lung diseases (3)?

A
47
Q

What is the mean survival of IPF?

A

2-3 yrs

48
Q

A characteristic feature of advanced disease is irreversible fibrosis. How would you tell if it is irreversible.
The answer to this question is also the main prognostic factor in ILD

A

Non-responsiveness to steroids (poor prognostic factor)

49
Q

What is the honeycombing indicate in an HRCT of ILD?

A

cystic air/ alveoli

50
Q

What is a granuloma?

A

A granuloma is a tiny cluster of white blood cells and other tissue.