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Respiratory > Interstitial and Granulomatous Lung Disease > Flashcards

Interstitial and Granulomatous Lung Disease Flashcards

1
Q

What is sarcoidosis?

A

A multisystem inflammatory disease of unknown aetiology which is characterised by non-necrotising granulomatous inflammation and predominantly affects the lungs and intrathoracic lymph nodes

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2
Q

Sarcoidosis is a diagnosis of exclusion. T/F?

A

True

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3
Q

What systemic symptoms occur in sarcoidosis?

A 
Fever
Anorexia
Fatigue
Night sweats
Weight loss
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4
Q

What lung symptoms occur in sarcoidosis?

A 
Dyspnoea on exertion
Cough
Chest pain
Wheezing
Haemoptysis
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5
Q

Other than lung findings and systemic findings what other systems can be affected by sarcoidosis?

A 
Pulmonary findings
Dermatological manifestations
Ocular manifestations
Cardiac manifestations
Neurologic manifestations
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6
Q

What are the four radiographic stages of sarcoidosis?

A

1 - Bilateral hilar lymphadenopathy without infiltration
2 - Bilateral hilare lymphadenopathy with infiltration
3 - Infiltration alone
4 - Fibrotic bands, bullae, hilarious retraction, bronchiectasis and diaphragmatic tenting

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7
Q

The radiographic stages of sarcoidosis indicate disease chronicity or correlate with changes in pulmonary function. T/F?

A

False

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8
Q

What age of patients are affected with idiopathic pulmonary fibrosis?

A

> 50 years old

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9
Q

What are the signs of idiopathic pulmonary fibrosis?

A 
Breathlessness
Bibasilar crackles
Clubbing
Honeycombing
Peripheral interstitial pattern
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10
Q

What are the symptoms of idiopathic pulmonary fibrosis?

A 
Breathlessness which is worse with exercise
Hacking dry cough
Fatigue
Weakness
Appetite
Weight loss
Clubbing
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11
Q

What are the possible causes of pulmonary fibrosis?

A

Occupational and environmental - silicosis, asbestosis, hypersensitivity pneumonitis
Drug induced - amiodarone, nitrofurantoin, methotrexate, cocaine
Connective tissue disease - lupus, RA, scleroderma
Primary diseases - sarcoidosis, LAM
Idiopathic
Genetic

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12
Q

How is pulmonary fibrosis diagnosed?

A

High resolution CT

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13
Q

Extrinsic allergic alveolitis is mediated by which cells?

A

T cell mediated response

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14
Q

What are the symptoms of extrinsic allergic alveolitis?

A 
Flu-like illness
Cough
High fever/chills
Dyspnoea
Chest tightness
Malaise
Myalgia
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15
Q

In chronic extrinsic allergic alveolitis what signs/symtpoms occur?

A 
Dyspnoea in strain
Sputum culture
Fatigue
Anorexia
Weight loss
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16
Q

What is the presentation of extrinsic allergic alveolitis?

A

Small <5mm opacities throughout both lungs, sometimes sparing the apices and bases
There is airspace disease which is usually seen as ground glass opacities and fine reticulation

17
Q

What are the characteristic of idiopathic pulmonary fibrosis on CXR?

A

Bibasal reticular abnormalities with a ground glass appearance

18
Q

What are the charaterstics of idiopathic pulmonary fibrosis on high resolution CT?

A

Subpleural reticular abnormalities
Honeycombing
Traction bronchiectasis

19
Q

‘Honeycombing’ can be seen on imaging of the lung in idiopathic pulmonary fibrosis. What does this mean?

A

Thick walled cysts 0.5 - 2cm in diameter in respiratory and terminal bronchioles

20
Q

What is the appearance of idiopathic pulmonary fibrosis on histology?

A

Dense interstitial fibrosis predominantly in the sub-pleural region
Destruction of normal lung architecture
Cystically dilated air spaces and intervening fibrous tissue
Inflammatory infiltrate of lymphocytes, plasma cells and intra-alveolar macrophages
Few fibroblastic foci

21
Q

Describe the pathogenesis of idioapthic pulmonary fibrosis.

A

Repeated cycles of epithelial activation and injury by an unknown agent. Abnormal activation of epithelial cells leads to a dysrgeulated repair process which causes the formation of fibroblastic foci and promotes fibrosis.

22
Q

What cytoline released from damaged epithelial cells in idiopathic pulmonary fibrosis leads to the increased development of myofibroblasts?

A

TGF beta one

23
Q

What can be used to treat idiopathic pulmonary fibrosis?

A 
Pirfenidone
Nintedanib
Long term oxygen therapy
Diuretics
N-acetyl cysteine
Antibiotics
Lung transplantation
24
Q

How does pirfenidone act to slow lung function decline in idiopathic pulmonary fibrosis?

A

It is an antifibrotic and has anti-inflammatory effects. It is thought to suppress fibroblast proliferation.

25
Q

Nintedanib is used to slow lung function decline in idioapthic pulmonary fibrosis. What is its mechanism of action?

A

Intracellular inhibitor of multiple tyrosine kinases

26
Q

What is the median survival fo idiopathic fibrosis four the time of diagnosis?

A

3-5 years

Respiratory (10 decks)

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