Restrictive Lung Diseases and ILD Flashcards
What is the definition of a restrictive lung disease?
What are iatrogenic causes of ILD?
What are the types of IPF?
usual interstitial pneumonia
nonspecific interstitial pneumonia
cryptogenic organizing pneumonia
acute interstitial pneumonia
What are the types of granulomatous ILD?
Sarcoidosis
hypersensitivity pneumonitis (organic dust)
farmer’s lung (actinomyces, hay, mold exposure)
Bird fancier’s lung - exposure to avian proteins
Tb
What CTDs cause lead to ILD?
List the types/ causes of ILD.
Other than ILD, what are some causes of restrictive lung diseases?
Classify all the causes of restrictive lung disease.
What gender is more likely to get the following restrictive lung diseases:
1. Sarcoidosis?
2. IPF?
- F>M
- M>F
What is the most common type of IPF (Give the full name)? What would you expect on histology and HRCT?
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)
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
What are the RFs for ILD?
What is the most common presentation of sarcoidosis and what symptoms would the patient have?
Lofgren’s Syndrome
*Perihilar lymphadenopathy
What symptoms would you like to elicit from a history of sarcoidosis?
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?
Bibasal fine end-inspiratory crackles
“Velcrow-like”
What are the 3Cs of ILD?
Clubbing
Cough (dry)
Crackles Bibasal fine end-inspiratory crackles
Outline the questions you would ask in a history for ILD.
This is a patient with sarcoidosis. What does this image show?
Lacrimal gland enlargement in sarcoidosis
This is a patient with sarcoidosis. What does this image show?
Parotid enlargement in sarcoidosis
This is a patient with sarcoidosis. What does this image show?
cutaneous lesions (lupus pernio)
This is a patient with sarcoidosis.
What does this image show?
Erythema nodosum
This is a patient with sarcoidosis.
What does this image show?
anterior uveitis
Just read this: Outline the exam findings you would expect to find in ILD.
What are the ddx for upper lobe fibrosis?
What are the ddx for Lower lobe fibrosis?
What are the ddx for Lung fibrosis?
DLCO is typically corrected for X. What is X and how does it affect DLCO?
Anaemia => must be corrected as it affects the gradient (less Hb => less diffusion gradient)
You are investigating a suspected ILD. Interpret the following results:
1. The DLCO comes back low.
2. The DLCO comes back normal.
- What is the DLCO and what does it assess?
What is the Gold-standard investigation for Lung fibrosis diagnosis?
Lung Biopsy and histology
What is the Gold-standard investigation for Sarcoidosis diagnosis?
Lung Biopsy and histology
What is the main HRCT findings for
1) IPF
2) Sarcoidosis
3) Asbestos
List the different methods of obtaining a lung biopsy
Bronchoscopy
EBUS - Endobronchial US
CT-guided biopsy
What are the diagnostic investigations for restrictive lung diseases?
What would you expect on the HRCT of the following pathologies:
1. IPF
2. Sarcoidosis
3. Asbestos
When is a surgical biopsy indicated in the setting of ILD? How is it done?
IF HRCT and Spirometry and not consistent & sarcoidosis
Bronchoscopy or EBUS
In the setting of ILD, what cause of ILD necessitates ordering histology of the biopsy? What would you expect to see?
Sarcoidosis - non-caseating granuloma
In a patient with ILD, what is the purpose of a 6 minute walk test?
What is the main finding on ECHO you are looking for in severe ILD?
Severe ILD => severe fibrosis => increasing pulmonary pressure => Pulmonary HTN
=> on ECHO you will see cor pulmonale (dilated IVC, RA…) + estimated Pulmonary wedge pressure
An ECHO can be used to estimate Pulmonary wedge pressure. How is this accurately measured?
Pulmonary Wedge Pressure is measured via a pulmonary artery catheter in the right side (@ the bifurcation)
What investigations would you order for a patient presenting with symptoms indicative of restrictive lung diseases?
(only mention directly relevant ones)
Outline the preventative strategies for restrictive lung diseases.
Outline the acute management of Restrictive lung diseases.
O2 and steroids (best for inflammatory cases –> all ILD EXCEPT FOR IPF.
Outline the chronic management of IPF. Include SEs of the medications
+
What is the role of these medications in the management of IPF
Outline the chronic management of Sarcoidosis. Include SEs of the medications.
What is the difference in etiology between hypersensitivity pneumonitis and pneumoconiosis?
Hypersensitivity pneumonitis from organic dust
Pneumoconiosis from inorganic dust
Outline the acute and chronic management of Inhalation ILD.
Outline the chronic management of chest wall disease
If needed, based on 6 minute walk test for portable O2
Outline the full management of a patient with restrictive lung disease.
What are the complications of restrictive lung diseases (3)?
What is the mean survival of IPF?
2-3 yrs
A characteristic feature of advanced disease is irreversible fibrosis. How would you tell if it is irreversible.
Non-responsiveness to steroids (poor prognostic factor)
The answer to this question is also the main prognostic factor in ILD
What is the honeycombing indicate in an HRCT of ILD?
cystic air/ alveoli
What is a granuloma?
What is it’s role?
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.
