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GCUH FRACP Writtens 2020 > Respiratory > Flashcards

Respiratory Flashcards

1
Q

Name 4 organisms that commonly cause respiratory infections in patients with cystic fibrosis.

A

Staph aureus

Pseudomonas aeruginosa

Burkholderia cepacia

Aspergillus

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2
Q

What is the Epworth Sleep Scale out of, and what is considered normal?

A

Out of 24; up to 10 is normal

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3
Q

What are the three key features of obstructive sleep apnoea syndrome?

A

Snoring

Apnoeas

Daytime somnolence

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4
Q

How do you distinguish obstructive sleep apnoea from central sleep apnoea?

A

CSA - periods of absence of respiratory effort, versus attempts to overcome obstructed upper airway

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5
Q

What happens in REM sleep that results in less use of respiratory ventilatory muscles and decreased sats?

A

Skeletal muscle paralysis - this also stops you acting out your dreams during REM sleep

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6
Q

Name 8 conditions associated with restless leg syndrome.

A

Iron deficiency

ADHD

Coeliac disease

COPD

Depression/panic disorders

Fibromyalgia

Parkinson’s disease

Multiple sclerosis

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7
Q

Name 5 (groups of) medications that can cause restless legs syndrome?

A

SSRIs

Neuroleptics

Lithium

Beta blockade

Dopamine antagonists

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8
Q

Following conservative treatments, what medications can you give for restless legs syndrome? Name 7.

A

Iron replacement

Levodopa

Ropinirole

Clonazepam

Tramadol

Oxycodone

Gabapentin

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9
Q

What is the pathophysiology of narcolepsy, and what are the two different types?

A

Acquired deficiency of orexin (hypocretin) in key hypothalamic neurons

Type 1 - narcolepsy with cataplexy

Type 2 - narcolepsy without cataplexy

Diagnosed by REM intrusions on PSG

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10
Q

How is narcolepsy treated? Name 3 drugs.

A

Modafinil - treats narcolepsy but not cataplexy

Methylphenidate - treats both

Dexamphetamine - treats both

Anti-cataplectic include venlafaxine and clomipramine

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11
Q

Name 7 absolute contraindications for receiving a lung transplant?

A

Recent history of malignancy

Major organ dysfunction

Chronic infection with virulent or resistant microbes that are poorly controlled pre-Tx

BMI > 35

Current or repeated non-adherence to treatment

Severely limited functional status with poor rehabilitation potential

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12
Q

Name two conditions where single lung transplant can be indicated.

A

ILD

COPD

Single lung transplant recipients will generally be older, and they will not have an infection.

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13
Q

Name 4 conditions where bilateral lung transplants can be indicated.

A

Cystic fibrosis

Bronchiectasis

Pulmonary hypertension

Severe infection

Bilateral lung transplant recipients will be young patients

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14
Q

When could COPD patients get listed for a lung transplant? Name 5 stipulations.

A

BODE 7 or greater

FEV 15-20%

3 or more exacerbations in one year

One episode of acute hypercapnic respiratory failure

Moderate to severe pH

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15
Q

When could pulmonary fibrosis patients be listed for a lung transplant? Provide 4 stipulations.

A

Decline in FVC >10% and DLCO >15% in the prior 6 months

Development of pulmonary hypertension

Hospitalisation because of respiratory decline, acute exacerbation or pneumothorax

Significant exercise associated desaturation or requirement for oxygen

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16
Q

When could pulmonary vascular disease patients be listed for a lung transplant? Provide 3 stipulations.

A

NYHA functional class III or IV despite escalating pulmonary vasodilator therapy

Refractory or progressive right heart failure

Right heart catheter measurements of mean right arterial pressure >15 mmHg, cardiac index <2 litres/minute/m2 and mean PAP >50 mmHg

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17
Q

When could cystic fibrosis patients be listed for a lung transplant? Provide 7 stipulations.

A

Frequent hospitalisation

FEV1 <30% of predicted especially if rapid downward trajectory

Increasing antibiotic dependence or resistance

Life-threatening haemoptysis or pneumothorax

Requirement for non-invasive ventilation

Development of pulmonary hypertension

PCO2 > 50 mmHg and/or pO2 <60 mmHg (need for NIV)

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18
Q

What is the greatest limitation to survival in lung transplant patients?

A

Chronic allograft lung dysfunction

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19
Q

Which hypoventilation syndrome has an association with CVD?

A

Central sleep apnoea

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20
Q

Name 4 risk factors for developing CTEPH

A

Pro-coagulant states (antithrombin, protein C and S deficiency)

Lupus anticoagulant and APS

Blood groups A, B and aB

Splenectomy

CTEPH patients require lifelong anticoagulation

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21
Q

What is the definitive treatment for CTEPH?

A

Pulmonary endarterectomy

Procedural mortality is 2-5%, and the operation should be done at an expert centre where 20/year are performed

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22
Q

What sweat chloride test result is diagnostic of cystic fibrosis?

A

> 60 mmol/L

40-60 is in between normal and abnormal

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23
Q

What is the definitive test for primary ciliary dyskinesia?

A

Examination of ciliary ultrastructure under electron microscopy

Expect to see abnormal ultrastructure and slow ciliary beat frequency

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24
Q

How is cystic fibrosis diagnosed?

A

Clinical features of CF or family history of CF

AND EITHER

2 CF-causing mutations OR

Positive sweat chloride on 2 occasions OR

Nasal potential difference tracing typical for CF

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25
Q

What is nasal potential difference?

A

Test for CF

Voltage correlates with movement of Na across a cell membrane; amiloride blocks epithelial Na channels; isoprotenerol stimulates CFTR

CF patients have:

A more negative baseline potential difference

Larger inhibition after addition of amiloride

Little or no change with addition of Cl free solution or isoprotenerol

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26
Q

What is the inheritance of cystic fibrosis, and what is the most commonly involved gene?

A

Autosomal recessive

Deletion on long arm chromosome 7

Delta F508

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27
Q

What is ivacaftor?

A

Treatment for CF with gene mutation G551D

Class 3 defect - CFTR reaches cell surface but channel opening is impaired

Class 4 defect - decreased conductance

Ivacaftor is a small molecule potentiator of the CFTR channel

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28
Q

What is lumacaftor?

A

CFTR corrector

Used in combination with ivacaftor for homozygous Phe508del

Small improvement in FEV1 but significant decrease in pulmonary exacerbations

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29
Q

What is the benefit of macrolides in cystic fibrosis?

A

Inhibits biofilm production

Modifies mucous production

Suppresses inflammatory markers

Indicated for CF and non-CF bronchiectasis with pseudomonas or frequent exacerbators without pseudomonas

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30
Q

What constitutes significant bronchodilator reversibility on spirometry?

A

Improvement of 12% and/or 200ml

31
Q

What does exhaled nitric oxide on PFTs help to indicate?

A

Whether or not there is inflammation present that might be responsive to steroids

Correlates with Th2 inflammation and sputum eosinophilia

32
Q

What 4 parameters are used in the GOLD ABCD assessment tool for COPD?

A

FEV1 (predicted) - 1 is >80, 2 is 50-79, 3 is 30-49, 4 is <30

Exacerbation Hx - A or B is 0 or 1 (not leading to hospital admission), C or D is >2 or >1 leading to hospital admission

mMRC questionnaire - A or C or 0 or 1, B or D is >2

CAT questionnaire - A or C is CAT < 10, B or D is CAT >10

33
Q

What are the 4 parameters used for the BODE index in COPD?

A

BMI

Obstruction - FEV1

Dyspnoea - mMRC dyspnoea scale

Exercise capacity - six-minute walk distance

34
Q

Which gene is responsible for the onset of alpha-1-antitrypsin deficiency?

A

SERPINA1

35
Q

What is the pathophysiology of alpha-1-antitrypsin deficiency?

A

Failure to inhibit neutrophil elastase in lung

Build up of AAT polymers in liver cells

36
Q

When is long term oxygen therapy indicated for COPD patients?

A

Non-smoking stable COPD patients who have:

PaO2 < 55

PaO2 < 55-59 with evidence of pulmonary hypertension or polycythaemia

Note - no evidence of improved outcomes in COPD patients with moderate hypoxia

37
Q

Name 5 benefits of pulmonary rehabilitation.

A

Improved exercise capacity

Reduced perceived intensity of breathlessness

Improved health-related quality of life

Reduced hospitalisation and hospital days

Reduced anxiety and depression in COPD

38
Q

Name 7 bacteria that can be seen in bacterial exacerbations of COPD.

A

H influenza

Strep pneumoniae

M catarrhalis

Mycoplasma

Chlamydia

In more severe disease - Staph aureus, Pseudomonas

39
Q

When would you consider referral for lung transplants in COPD patients? Name 5 considerations.

A

When the majority of the following are present…

Progressive symptoms, despite maximal treatment including medication, pulmonary rehabilitation and oxygen therapy

Patient is not a candidate for endoscopic or surgical lung volume reduction surgery

BODE index of 5-6

PaCO2 > 50 and/or PaO2 < 60

FEV1 < 25% predicted

40
Q

What is a DECAF score, and what are its constituent parts?

A

Predictor of in-hospital mortality from COPD exacerbations

Dyspnoea

Eosinopaenia

Consolidation

Acid anemia

Atrial fibrillation

Score of 0-1 low risk; 2 intermediate risk; 3-6 high risk

41
Q

Which vaccinations are recommended for COPD patients?

A

Influenza

Pneumococcal

42
Q

When would you consider adding an ICS to treatment for COPD?

A

If FEV1 < 50%

2 or more hospitalisations within a year

All despite LABA and LAMA therapy

43
Q

Name 4 viruses that can cause viral exacerbations of COPD

A

Influenza

Rhinovirus

RSV

Metapneumovirus

44
Q

Name the main 3 cell types and cytokines that are involved in the pathogenesis of asthma.

A

Cells

  • Th2 cells
  • mast cells
  • eosinophils

Cytokines

  • IL-4
  • IL-5
  • IL-13
45
Q

What are the pathological processes that occur in asthma? Name 5.

A

Mucosal inflammation

Mucoid exudate

Goblet cell metaplasia

Epithelial basement membrane thickening

Smooth muscle hypertrophy, hyperplasia and contraction

46
Q

How is asthma diagnosed on spirometry?

A

FEV1 increase >200ml and >12% from baseline post bronchodilator

47
Q

What does an increased exhaled nitric oxide test suggest on spirometry?

A

Suggests steroid-responsive airway disease.

48
Q

What are the stages of treatment for asthma?

A

Stage 1 - SABA as required

Stage 2 - Low dose regular preventer (ICS) + SABA as required

Stage 3 - Low dose combo regular preventer (LABA + ICS) + reliever as required

  • budesonide/formoterol (low dose) maintainence-and-reliever therapy
  • ICS/LABA combo (low dose) as maintenance

Stage 4 - High dose combo regular preventer (LABA + ICS) + reliever as required

  • budesonide/formoterol maintenance (medium dose) and reliever (low dose) therapy
  • ICS/LABA combo (moderate-high dose) as maintenance therapy

Stage 5 - specialised treatments

49
Q

What is SMART therapy for asthma?

A

Single Inhaler Maintenance and Reliever Therapy (SMART)

Budesonide/formoterol combination inhaler, 2 puffs BD and PRN

Used in moderate-to-severe asthma

50
Q

What is omalizumab, and what stipulation is required to use it?

A

Anti-IgE - mAb that binds the Fc portion of IgE to prevent if from activating the high-affinity IgE receptor on mast cells, basophils and dendritic cells

Used in asthma principally

Baseline IgE needs to be <1300 IU/mL, otherwise too much IgE to bind

51
Q

What is the main adverse effect of omalizumab?

A

Anaphylactoid reactions

52
Q

What is mepolizumab?

A

Anti IL-5 - mAb that reduces production and survival of eosinophils

Used in severe asthma; reduces exacerbations and has a steroid-sparing effect

53
Q

Name 2 drugs that can be used for the treatment of IPF?

A

Pirfenidone

Nintedanib

54
Q

Name 4 features on HRCT consistent with usual interstitial pneumonia

A

Pleural/subpleural predominance

Traction bronchiectasis

Reticular shadowing

Honeycombing

55
Q

Name 6 conditions associated with lymphocytic interstitial pneumonia.

A

Sjögren’s syndrome

Rheumatoid arthritis

HIV infection

SLE

Myasthenia Travis

Chronic active hepatitis infections

56
Q

Name 2 features on HRCT of lymphocytic interstitial pneumonia.

A

Discrete peribronchovascular cysts

Varying degrees of ground glass opacities

Note - lot of overlap with NSIP, gold standard of diagnosis is biopsy

57
Q

What are the two different types of NSIP that can be seen?

A

Cellular

Fibrotic

58
Q

What features would you expect to see on HRCT with hypersensitivity pneumonitis? Name 3 features.

A

Upper zone predominant

Fine nodules (starry sky) with airway involvement

Inspiratory/expiratory scans demonstrate segmental air trapping and hence mosaic ventilation patterns

59
Q

What are the features of cryptogenic organising pneumonia on HRCT?

A

Pleurally based

Dense consolidation in triangular, pleural areas

Air bronchograms

Peribronchovascular densities

60
Q

What is pulmonary lymphangioleiomyomatosis?

A

A diffuse bilateral cystic disease that is either sporadic or associated with tuberous sclerosis complex

Associated with angiomyolipomas of the kidneys and meningiomas

Management is with mTOR inhibitors or lung transplantation

61
Q

Name 2 radiographic patterns on HRCT associated with smoking.

A

RBILD (respiratory bronchiolitis-associated interstitial lung disease)

DIP (desquamative interstitial pneumonia)

DIP is a later presentation and likely represents the end stage of RBILD

62
Q

What are the features on HRCT you would expect to see with RBILD (respiratory bronchiolitis-associated interstitial lung disease)? Name 5.

A

No zonal predominance

Bilateral

Prominent ground glass

Bronchial wall thickening

Coexisting centrilobar emphysema

63
Q

Name 3 epidemiological factors associated with Langerhans cell histocytosis X (LCHC).

A

Young adults (20-40)

Smokers

Male > female

64
Q

Name 4 features/disease associations with Langerhans cell histocytosis X.

A

Diabetes insipidus

Lytic bone lesions

Lymphoma

Pituitary hormone deficiencies

65
Q

Where does CT prove to be more beneficial than PET scanning when looking for malignancy lung nodules?

A

When nodules are small (<0.7cm)

Also with bronchoalveolar Ca and carcinoid, as these exhibit low uptake

66
Q

Name 2 confounding factors that can result in increased nitric oxide upon exhalation testing.

A

URTI

Air pollution exposure

67
Q

Name 4 confounding factors that can result in decreased nitric oxide upon exhalation testing.

A

Steroids

Leukotriene receptor antagonists

Exercise

Smoking

68
Q

Name 2 bacteria that are strongly linked to sarcoidosis.

A

Mycobacteria

Proprionibacteria

69
Q

What are the characteristic findings on CT of sarcoidosis? Name 4.

A

Mediastinal and hilar lymphadenopathy

Lung disease with upper lobe predominance

Peribronchial irregularities

Subpleural micronodules

70
Q

What is found on histology for sarcoidosis?

A

Non-caseating granulomas

Occasionally necrosis

71
Q

What is the treatment for sarcoidosis.

A

50-60% of cases spontaneously remit

Corticosteroids for severe or progressive disease

Immunosuppressive treatment if steroid failure

  • methotrexate is slow-acting, may take up to 6 months for benefit
  • cyclophosphamide is treatment of choice for CNS sarcoidosis
  • antimalarials have a role in skin, splenic involvement and hypercalcaemia
72
Q

What are the characteristic features of bronchiectasis on HRCT? Name 3.

A

Airway dilatation

Bronchial wall thickening

Mucous plugging of airways - with tree-in-bud pattern

73
Q

What is the alveolar gas equation?

A

paO2 = FiO2 (pATM - pH2O) - (paCO2 (1 - FiO2 (1 - RER)))/RER

paO2 = alveolar partial pressure of O2

FiO2 = fraction of inspired gas that is O2 (0.21)

pATM = prevailing atmospheric pressure (usually 760 at sea level)

pH2O = water vapour pressure (usually 47 at sea level)

paCO2 = partial pressure of carbon dioxide

RER = respiratory exchange ratio (0.8)

Simplified version, putting the numbers in, assuming sea level: 150 - (paCO2 x 1.25)

74
Q

What does peptostreptococcus typically cause?

A

Empyemas - anaerobic infection

Foul tasting sputum is characteristic

GCUH FRACP Writtens 2020 (14 decks)

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