Interstitial lung disease, fungal lung disease, bronchiectasis and cystic fibrosis Flashcards

1
Q

Give 5 presenting features of interstitial lung disease

A
Dyspnoea on exertion
Dry cough
Cyanosis
Fine crackles
Restrictive spirometry
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2
Q

Give 4 examples of pneumoconioses

A

Silicosis
Asbestosis
Berylliosis
Coal worker’s pneumoconiosis

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3
Q

Give 3 types of extrinsic allergic alveolitis

A

Pigeon fancier’s lung
Farmer’s lung (inhalation of fungal spores)
Malt worker’s lung (inhalation of Aspergillus clavatus)

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4
Q

Give 7 causes of upper lung zone fibrosis

A
Berylliosis
Radiation
Extrinsic allergic alveolitis
Ankylosing spondylitis
Silicosis
Tuberculosis
Sarcoidosis
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5
Q

Give 6 causes of lower lung zone fibrosis

A
Bronchiectasis
Rheumatoid arthritis
Asbestosis
Idiopathic pulmonary fibrosis
Nitrofurantoin
Scleroderma
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6
Q

Give 4 extrapulmonary presenting features of sarcoidosis

A

Swinging fever
Polyarthralgia
Lupus pernio
Hypercalcaemia

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7
Q

What causes hypercalcaemia in sarcoidosis?

A

Macrophages activating vitamin D

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8
Q

Give 5 indications for prednisolone in the management of sarcoidosis

A
Symptomatic stage 2 or 3 disease
Eyes involved
Heart involved
CNS involved
Hypercalcaemia
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9
Q

What is the 5 year survival of idiopathic pulmonary fibrosis?

A

50%

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10
Q

What is the gold standard investigation for idiopathic pulmonary fibrosis?

A

High resolution CT

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11
Q

What is the mechanism of pirfenidone?

A

Inhibits TGF-beta and limits fibroblast proliferation

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12
Q

What is the mechanism of nintedanib?

A

Tyrosine kinase inhibitor

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13
Q

Give 2 drugs used in the management of idiopathic pulmonary fibrosis

A

Pirfenidone

Nintedanib

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14
Q

Give 3 side effects of pirfenidone

A

Nausea
Vomiting
Photosensitive rash

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15
Q

What is the only definitive treatment for idiopathic pulmonary fibrosis?

A

Lung transplant

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16
Q

Give 5 ways in which Aspergillus affects the lung

A
Asthma
Extrinsic allergic alveolitis
Allergic bronchopulmonary aspergillosis
Aspergilloma
Invasive aspergillosis
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17
Q

Give 3 key investigations in the diagnosis of allergic bronchopulmonary aspergillosis

A

Sputum culture
Aspergillus skin test
Aspergillus-specific IgE radioallergosorbent test

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18
Q

What is the first line management of allergic bronchopulmonary aspergillosis?

A

Prednisolone

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19
Q

Give 2 options for the second-line management of allergic bronchopulmonary aspergillosis

A

Bronchodiltors

Itraconazole

20
Q

Give 2 conditions which can cause formation of a lung cavity, which can become an aspergilloma

A

Tuberculosis

Sarcoidosis

21
Q

Give 4 presenting features of aspergilloma

A

Cough
Haemoptysis
Lethargy
Weight loss

22
Q

What does an aspergilloma look like on CXR?

A

Round opacity within a cavity

23
Q

Give 3 key investigations for aspergilloma

A

Sputum culture
Aspergillus skin test
Serum precipitins

24
Q

Should you treat asymptomatic aspergillomas?

A

No

25
Q

Give 2 indications for surgery to remove aspergilloma

A

Solitary symptomatic lesions
OR
Massive haemoptysis

26
Q

GIve 2 non-surgical options for aspergilloma

A

Oral itraconazole

CT-guided local instillation of amphotericin paste

27
Q

Give 2 risk factors for invasive aspergillosis

A

Immunosuppression

Broad-spectrum antibiotics

28
Q

What is the gold standard diagnostic test for invasive aspergillosis?

A

Biopsy/autopsy

29
Q

Give 2 investigations which may be useful (but not diagnostic) in the diagnosis of invasive aspergillosis

A

CT chest

Serum galactomannan

30
Q

What is the first line management of invasive aspergillosis?

A

Voriconazole

31
Q

What is the pathogenesis of bronchiectasis?

A

Chronic inflammation leads to permanent airway dilatation

32
Q

What is Kartagener syndrome?

A

An inherited disorder of the cilia in the respiratory tract and spermatic duct/oviduct

33
Q

How does Kartagener syndrome present?

A

Sinusitis
Bronchiectasis
Infertility
Situs inversus

34
Q

Give 5 presenting features of bronchiectasis

A
Cough (worse when lying down)
Clubbing
Dyspnoea
Wheeze
Coarse crackles
35
Q

What CXR finding suggests bronchiectasis?

A

“Tram track” shadows

36
Q

Is bronchiectasis restrictive or obstructive?

A

Obstructive

37
Q

What is the gold standard investigation for bronchiectasis?

A

High resolution CT

38
Q

Which vaccinations should be given to people with bronchiectasis?

A

Annual influenza and one-off pneumococcal vaccine

39
Q

Which antibiotics can be given in exacerbations of bronchiectasis until sputum culture results return?

A

Amoxicillin (first line)
Clarithromycin
Doxycycline

40
Q

What is the indication for long-term antibiotics in patients with bronchiectasis?

A

3 or more exacerbations per year

41
Q

Give 2 organisms which cause recurrent chest infections in patients with cystic fibrosis

A

Staphylococcus aureus

Pseudomonas aeruginosa

42
Q

Give 3 conditions which can cause a false positive cystic fibrosis sweat test

A

Malnutrition
Adrenal insufficiency
Hypothyroidism

43
Q

What can cause a false negative cystic fibrosis sweat test?

A

Skin oedema as a result of pancreatic exocrine insufficiency causing hypoproteinaemia

44
Q

Orkambi is used for cystic fibrosis with which mutation?

A

Delta-F508 mutation

45
Q

Orkambi is a combination of which 2 drugs?

A

Lumacaftor

Ivacaftor

46
Q

What is the mechanism of action of lumacaftor?

A

Chaperone protein - increases number of CFTR proteins at the cell surface

47
Q

What is the mechanism of action of ivacaftor?

A

Potentiates opening of the CFTR channel