Restrictive Lung Diseases and ILD

Question 2

What is the definition of a restrictive lung disease?

Answer 2

Question 3

What are iatrogenic causes of ILD?

Answer 3

Question 4

What are the types of IPF?

Answer 4

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

Question 5

What are the types of granulomatous ILD?

Answer 5

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

Question 6

What CTDs cause lead to ILD?

Answer 6

Question 7

List the types/ causes of ILD.

Answer 7

Question 8

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

Answer 8

Question 9

Classify all the causes of restrictive lung disease.

Answer 9

Question 10

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

Answer 10

1. F>M
2. M>F

Question 11

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

Answer 11

Question 12

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)

Answer 12

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

Question 13

What are the RFs for ILD?

Answer 13

Question 14

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

Answer 14

Lofgren’s Syndrome
*Perihilar lymphadenopathy

Question 15

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

Answer 15

Question 16

You are asked to auscultate the lung of a patient who has been taking methotrexate for the past 3 years to control their rheumatoid arthritis. What is the most important finding to look for?

Answer 16

Bibasal fine end-inspiratory crackles
“Velcrow-like”

Question 17

What are the 3Cs of ILD?

Answer 17

Clubbing
Cough (dry)
Crackles Bibasal fine end-inspiratory crackles

Question 18

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

Answer 18

Question 19

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

Answer 19

Lacrimal gland enlargement in sarcoidosis

Question 20

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

Answer 20

Parotid enlargement in sarcoidosis

Question 21

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

Answer 21

cutaneous lesions (lupus pernio)

Question 22

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

Answer 22

Erythema nodosum

Question 23

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

Answer 23

anterior uveitis

Question 24

Just read this: Outline the exam findings you would expect to find in ILD.

Answer 24

Question 25

What are the ddx for upper lobe fibrosis?

Answer 25

Question 26

What are the ddx for Lower lobe fibrosis?

Answer 26

Question 27

What are the ddx for Lung fibrosis?

Answer 27

Question 28

DLCO is typically corrected for X. What is X and how does it affect DLCO?

Answer 28

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

Question 29

You are investigating a suspected ILD. Interpret the following results: 1. The DLCO comes back low. 2. The DLCO comes back normal. 3. What is the DLCO and what does it assess?

Answer 29

Question 30

What is the Gold-standard investigation for Lung fibrosis diagnosis?

Answer 30

Lung Biopsy and histology

Question 31

What is the Gold-standard investigation for Sarcoidosis diagnosis?

Answer 31

Lung Biopsy and histology

Question 32

What is the main HRCT findings for 1) IPF 2) Sarcoidosis 3) Asbestos

Answer 32

Question 33

List the different methods of obtaining a lung biopsy

Answer 33

Bronchoscopy EBUS - Endobronchial US CT-guided biopsy

Question 34

What are the diagnostic investigations for restrictive lung diseases?

Answer 34

Question 35

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

Answer 35

Question 36

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

Answer 36

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

Question 37

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

Answer 37

Sarcoidosis - non-caseating granuloma

Question 38

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

Answer 38

Question 39

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

Answer 39

Severe ILD => severe fibrosis => increasing pulmonary pressure => Pulmonary HTN => on ECHO you will see cor pulmonale (dilated IVC, RA...) + estimated Pulmonary wedge pressure

Question 40

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

Answer 40

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

Question 41

What investigations would you order for a patient presenting with symptoms indicative of restrictive lung diseases? (only mention directly relevant ones)

Answer 41

Question 42

Outline the preventative strategies for restrictive lung diseases.

Answer 42

Question 43

Outline the acute management of Restrictive lung diseases.

Answer 43

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

Question 44

Outline the chronic management of IPF. Include SEs of the medications + What is the role of these medications in the management of IPF

Answer 44

Question 45

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

Answer 45

Question 46

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

Answer 46

Hypersensitivity pneumonitis from organic dust Pneumoconiosis from inorganic dust

Question 47

Outline the acute and chronic management of Inhalation ILD.

Answer 47

Question 48

Outline the chronic management of chest wall disease

Answer 48

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

Question 49

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

Answer 49

Question 50

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

Answer 50

Question 51

What is the mean survival of IPF?

Answer 51

2-3 yrs

Question 52

A characteristic feature of advanced disease is irreversible fibrosis. How would you tell if it is irreversible.

Answer 52

Non-responsiveness to steroids (poor prognostic factor) The answer to this question is also the main prognostic factor in ILD

Question 53

What is the honeycombing indicate in an HRCT of ILD?

Answer 53

cystic air/ alveoli

Question 54

What is a granuloma? What is it's role?

Answer 54

A granuloma is a localized collection of activated macrophages surrounded by lymphocytes and fibroblasts. this occurs secondary to persistent inflammation and it's role is to contain the inflammation/infection rather than eliminate it.