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FRACP Exam > Resp > Flashcards

Resp Flashcards

1
Q

What are the CT findings of bronchiectasis?

A
  • Bronchoarterial ratio >1
    • Lack of airway tapering
    • Airway visibility within 1 cm of costal pleural surface or
      • Touching mediastinal pleura.
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2
Q

What are the indicators for the bronchiectasis severity index / a worse QoL and higher mortality in bronchiectasis?

A

FEV1 % (<80% predicted)
Number of exacerbations and number of hospital admissions in the past 2 years
MRC breathlessness score
Pseudomonas colonisation
Radiological severity
Age (>50 , >70, > 80)
BMI - low BMI = bad

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3
Q

How do you manage bronchiectasis?

A

Step 1 : Treat cause, PT for airway clearance
Step 2: If >3 exac/year - PT + mucoactive treatment
Step 3: If >3 exac/year despite 1 + 2 - Long term antipseudomonal if colonised, Long term macrolide
Step 4: If >3 exac/year despite 1+2+3 - Long term macrolide + inhaled abx
Step 5: >5 exac/year despite above - regular IV antibioitcs every 2-3 motnhs.

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4
Q

Why do we use macrolides for exacerbation prevention in bronchiectasis

A

Immunomodulatory.+ antiboitic
Theuy reduce frequency of exac and time to first exac, and QoL.
No impact on FEV1

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5
Q

What are the outcomes if Bronchiectasis + Pseudomonas colonisation

A

Worse symptoms, worse radiological findings, increased disease sevierity, more inflammation, worse overall outcomes

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6
Q

What is the pattern of inheritance in cystic fibrosis?

A

Autosomal recessive

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7
Q

What is the mutation in cystic fibrosis?

A

Delta F508 mutation . The regulator gene CFTR for the transmembrane transporter of Na and Cl in the ciliary epithelium

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8
Q

What is required for diagnosis of CF?

A

1 or more of:
- Chronic pulmonary disease
- Chronic sinusitis
Salt loss syndromes
Obstructive azoospermia
History of CF in a sibling
Positive newborn screening
AND 1 of:
Elevated sweat chloride
2 CFTR gene variants known to cause CF
Abn in the nasal potential differential testing

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9
Q

Can males with CF reproduce?

A

No. 95% infertile
Absent vas deferens + defects in sperm transport. Spermatogenesis not affectes

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10
Q

what is aquagenic wrinkling a sign of?

A

CF - CFTR dysfunction

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11
Q

What agents are used in “mucoactive therapy”

A

Inhaled dornase alpha, inhaled hypertonic saline, PEP

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12
Q

What effect does macrolides have in CF?

A

Improved FEV1, reduced exacerbations.

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13
Q

What are the bugs of significance in CF?

A

Burkholderia cepacia complex - worse transplant outcomes, shortened survival, accelerated decline in lung function
Non-tuberculous mycobacteria in 10-20% - worse transplant outcomes

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14
Q

What is a class I CFTR mutation + how do you treat?

A

Nonsense mutation - no functional CFTR protein
Gene therapy

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15
Q

What is a class II CFTR mutation ?

A

CFTR protein is misfolded CFTR traficking defect- F508del
gene therapy

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16
Q

What is a class III CFTR mutation?

A

CFTR protein is created, reaches the cell surface but defective channel regulationy - G551D

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17
Q

What is a class IV CFTR mutation ?

A

Decreased channel conductance

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18
Q

What is a class V CFTR mutation + how do you treat?

A

CFTR is created in insufficient quantities.
Gene therapy

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19
Q

What is Ivacaftor?

A

CFTR potentiator
Increases chloride secretion and reduces excessive sodium and fluid absorption.
Works on Class IV
Prevents airway dehydration, improves cilia motility and improved FEV 1 by 10%. ONLY for G551D

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20
Q

What are Tezacaftor/lumacaftor?

A

Correctores/potentiators of CFTR misfolding so good in class II defects

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21
Q

What is the best treatemtn for homozygous F508Del in CF ?

A

Lumacaftor and Ivocaftor combined.
Lumacaf - improves misfolding
Ivacaf - improved channel gating activity
Reduced exacerbatiosn by 39%

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22
Q

What is the benefits of tripple therapy in CF? Elexacaftor-Tezacaftor-Ivacaftor

A

For F508Del mutation
- Increased chloride transport
Improved QoL, imrpvoed FEV1, weight gain, reduced long term oxygen, reduced need for NIV, reduced PEG feeding requirement

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23
Q

What are indications for lung transplant in CF?

A

FEV1 <30%
Rapid decline in FEV1 despite optimal treatment
pO2 <60mmHg, pCO2 >50mmHg
Malnutrition and diabetes
Frequent exacerbations
Recurrent massive haemoptysis
Relapsing or complicated pneumothorax
ICU admission

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24
Q

What are contraindications (relative) for lung transplant in CF?

A

Age >65, clinically unstable, limited functional status without rehab potential, colonisation with Burkholderia cenocepacia, burkhodenia gladioli, or mycobacteria abscessus, disease not optimally treated

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25
Q

What is bronchiectasis?

A

Permanent dilation of bronchi and bronchioles due to destruction of airway muscles and elastic connective tissue

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26
Q

What are the immune effects of macrolides in chronic airway inflammation?

A

Mechanism of action of macrolides:
- Impair production of proinflammatoyr cytokines including TNF-a
- Inhibit neutrophil adhesion to cells
- Inhibit respiratory bursts of neutrophils
- Reduce mucus secretion from airways
Improve macrophage clearance of apoptotic cells
- Inhibit quorum sensing signals deceasing biofilm development (pseudomonas)

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27
Q

What level is diagnostic for a chloride test?

A

> 60mmol/l
Borderline 40-60mmol/l - these need to go on to CFTR genotype testing

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28
Q

What does Ivacaftor do?

A

CFTR potentiator - opens the channel of the protein

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29
Q

What does tezacaftor/Elexacaftor do?

A

CFTR corrector - moves the protein to the cell surface

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30
Q

What is distal intestinal obstruction syndrome?

A

A complication of CF - impaction of secretions in the ileocaecal junction
RIF faecal mass
can mimic appendicitis
Faeces in small bowel on AXR

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31
Q

What type of airway inflammation predominates in bronchiectasis?

A

Neutrophilic inflammation, occasionally eosinophilic

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32
Q

What are patients with primary immunodeficiency more at risk of in bronchiectasis?

A

Greater decline in lung function ( XLA, CVID)
Treat with IVIG which would slow decline in FEV

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33
Q

What is bronchiectasis overlap syndrome?

A

When patients have bronchiectasis in association with another connective tissue disorder eg RA - they have worse outcomes

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34
Q

What are risk factors for having a bronchiectasis exacerbation
?

A

IgG2 deficiency, chronic bacterial infection, resp viral infections, hs exacerbation

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35
Q

What is Ivacaftor monotherapy not useful in?

A

F508del mutation

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36
Q

What are the dianostic criteria for ABPA?

A

1 x predisposing condition ( CF, asthma) +
- Positive skin prick test or elevated IgE to aspergillus fumigatus AND
-Elevated IgE concentration ( >1000IU)
AND 2 of
- Radiology consistent with ABPA
- Total eosinophils >0.5 if steroid naive
- Positive aspergillis precipitants or elevated IgG to A. fumigatus

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37
Q

What are the radiological findings of ABPA?

A
  • Proximal cylindrical bronchiectasis
    • Mucus plugging
    • Tree in bud opacity
    • Atelectasis
    • Peripheral consolidation
    • Ground glass opacity
    • Mosaic attenuation with gas trapping
      CT is normal in ~20%
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38
Q

What is the treatment for ABPA exacerbation?

A

Pred 0.5mg/kg weaned over 3 months to supress the immune response to aspergillis
Long term treatment does not prevent relapse

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39
Q

How do you treat steroid dependent ABPA?

A

Anti-fungal
Itraconazole/voriconazole
Improved IgE, radiology and QoL and symptoms. Used for 16 weeks in patients who are steroid dependent.

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40
Q

What are the risks of use of itraconazole in APBA?

A

Itraconazole inhibits CYP3A4 increasing steroid concentration if on ICS - risk of cushings

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41
Q

CT findings of Nodules and cysts in the upper lung zones with recurrent pneumothorax is associated with what?

A

Pulmonary Langerhans cell histiocytosis

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42
Q

Pancoast tumours occur most commonly in what lung ca?

A

Squamous cell

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43
Q

What are the cut offs for mild, mod severe, very severe airflow obstruction?

A

Mild>70
Mod 60-69%
Severe 35-49%
Ver severe <35%

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44
Q

What do you do if spirometry suggests restrictive disease? (re4duced FVC, or reduced FEV1 and FCV)

A

Total lung volumes

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45
Q

What are spiro findings for fixed large airway obstruction ? (e.g tracheal stenosis)

A

Flat (plateau) expiratory loop AND inspiratory loop

46
Q

What are spiro findings for intrathoracic variable obstruction?

A

Flat expiratory loop, with normal inspiratory loop.
(INTRAthoracic effects EXPiratory loop)

47
Q

Whats the spiro findings for an extrathoracic variable obstruction?

A

Normal expiratory loop and flat Inspiratory loop
( EXTRAthoracic, INSPiratory loop)

48
Q

How do you calculate A-agradient at sea level ant normal temp?

A

A-a= (150-(1.25PaCO2))-PaO2)

49
Q

How do you calculate H+ from a ABG?

A

H= 24(PaCO2/HCO3)

50
Q

What is the ratio of HCO3 increasing to PCO2 increasing in acute and chronic respiratory acidosis?

A

Acute: HCO3 inc by 1mml/l for every 10mmHg PaCO2
Chronic HCO3 inc by 4mmol/L for every 10mmHg PaCO2

51
Q

What are the radiological findings of UIP?

A
  • Subpleural, basal predominantes
    • Honey combs
    • NOT ground glass - this is inflammatory
      Retucular abnormality
52
Q

What are the radiologic features of Non-specific interstitial pneumonia

A
  • Bilateral basal predominant ground glass opacification without honey combing or traction bronchiectasis
53
Q

What are the radiologic findings of organising pneumonia?

A

Consolidation, bronchovascula in distribution

54
Q

What are the radiologic findings of desquamative interstitial pneumonia

A

Ground glass with small cysts

55
Q

What are the radiologic findings of hyprsensitivity pneumonitis acute vs chronic?

A

Acute: Ground glass nodularity
Chronic: Fibrotic changes like UIP, with ground glass and gas trapping
Inspiratory/expiratory scans demonstrate segmented air trapping and hence mosaic ventalation patterns

56
Q

What adiologic pattern is associated with Idiopathic pulmonary fibrosis?

A

UIP (diagnosis requires exclusion of other causes of pulmonary fibrosis)

57
Q

What is the mechanism of action of pirfenidone?

A

acts through TGF-B and reduces fibroblas proliferation

58
Q

What is the MoA of nintedanib

A

Inhibits multiple TKIs (PDGF, VEGF, FGF)

59
Q

What are the benefits of Pirfenidone and nintedinib?

A

Reduce rate of decline of FVC and improve survival

60
Q

What are the SE of Pirfenidone?

A

Nausea and UGI side effects and photosensitivity rash

61
Q

What are the side effects of nintedanib

A

Diarrhoea, weight loss

62
Q

Features of lymphangioleiomyomatosis (LAM) ?

A

Combination of pulmonary cysts and renal angiomyolipoma in a young woman is a classic presentation

63
Q

What is “Crazy paving” on CT chest? + what does it indicate

A

refers to the appearance of ground-glass opacities with superimposed interlobular septal thickening and intralobular septal thickening.
Seen in Pulmonary alveolar proteinosis

64
Q

What defines an apnoea and hypopnoea?

A

Apnoea - decresed sats and reduced air flow by 90% >10s
Hypopnoea - Decreased airflow 30% and decresed sats by 3% >10s

65
Q

What finding is different in OSA vs OHS?

A

Always have high pCO2 in OHS, not always in OSA

66
Q

What are needed for dx OHS

A

Elevated pCO2 - during or immediately after sleep, BMI >35 and no other reason for pCO2

67
Q

What is the treatment of central sleep apnoea?

A

No evidence of Bipap or Cpap, if treat patients with reduced ejection fraction there is increased mortality

68
Q

How do you treat OHS?

A

CPAP with or without O2, BiPAP second line

69
Q

How many overweight people have OHS?

A

15% people with BMI >30, 50% BMI >50

70
Q

What is the mechanism of OHS?

A

Altered chemoresponsiveness, increased mechanical work, reduced lung volumes (ERV), and VQ mismatch.

71
Q

What electrolyte is associated with restless leg?

A

Iron

72
Q

What is narcolepsy?

A

Sleep latency test <10 min, REM sleep latency test < 30min and a multiple sleep latency test (MSLT) mean sleep latency <8min. REM sleep within 15min very suggestive

73
Q

What is cataplexy?

A

Sudden loss of motor power during the waekfulness due to REM intrusions

74
Q

What are the types of nacolepsy?

A

Type 1 - narcolepsy with cataplexy
Type 2 - Narcolepsy without cataplexy
Acquired deficiency of orexin

75
Q

How to treat narcolepsy?

A

T1 - Modafinil for narcolapsy, methylphenidate narc and cataplex

76
Q

What are the 5 classes of pulmonary HTN

A
  1. Pulmonary arterial HTN
  2. Pulmonary HTN due to Left heart disease
  3. Pulmonary HTN due to lung disease +/- hypoxia
  4. Pulmonary HTN due to pulmonary artery obstruction
    5.Pulmonary HTN with unclear and/or multifactorial mechanisms
77
Q

Whats the criteria for PAH?

A

mPAP >20mmHg
PAWP <15mmHg
PVR >2 WU

78
Q

What is the most common cause of type 1 Pul HTN?

A

Schistosomiasis ( Idiopathic is second)

79
Q

What gene is involved in hereditary PAH?

A

BMPR2 - bone morphogenetic protein
AD with incomplete penetrance
BMPR2 abnormalities result in proliferations of pulmonary vascular cells leading to vascular remodelling.

80
Q

What are the 3 vascular mediators of PAH?

A

Incr Endothelin- Vasoconstricter and mitogen
Decr NO - vasodilator and antiproliferative
Decr Prostacyclin - Vasodilator, antiproliferative and inhibits platelet function

81
Q

What do endothelin receptor A and B do?

A

Foundon smooth muscle, In combination cause vasocontriction
Endothelin B alone on vascular endothelium, they release NO - antiproliferative effect

82
Q

If patients are vasoreactive during a RHC, what med might they benefit from ?

A

If there is a drop in the mean pulmonary arterial pressure of >10mmHg to reach a mPAP of <40mmHg without a decrease in cardiac output then there will be benefit from adding in a calcium channel blocker.

83
Q

What meds act on the endothelin pathway in PH?

A

Selective endothelin receptor antagonist - Ambrisentan (ETA)
Dual endothelin receptor antagonight - ETA and ETB - Bosentan or macitentan

84
Q

What meds work on NO pathway in PH?

A

sGC stimulator - Riociguat, increases sensitivity of sGC to NO and stimulates NO receptor
PDE5 inhibitors - Sildenafil, tadalafil - increases intracellular cAMP and cGMP leading to pul vasodilation

85
Q

What meds work on the prostacyclin pathway

A

Prostacyclin analogues - epoprotenol, treprostinil, iloprost
Non-prostanoid IP receptor agonists - slecipag
These increase cAMP leading to vasodilation

86
Q

What things are in the ESC guidelines for risk assesment of PAH?

A

Clinical signs of RHF
progression of symptoms
Syncope
WHO functional class
6min walk distance
Pardiopul exercise testing
BNP levels
ECHO Right atrial area + pericardial effusion
Right atrial pressure

87
Q

What is normal pulmonary arterial wegde pressure?

A

<12

88
Q

What are the histological findings in PAH?

A

Intimal fibrosis, endothelial proliferation, medial thickening - smooth mucle cell hypertrophy and hyperplasia

89
Q

What is CTEPH?

A

Chronic thromboembolic pulmonary disease:
Mechanical obstruction of pul arteries by thrombus that doesnt resolve .
SOB >3months anticoag

90
Q

What conditions affect upper lobes?

A

Upper lobes (SCHART-S)

silicosis (progressive massive fibrosis), sarcoidosis
coal workers’ pneumoconiosis (progressive massive fibrosis)
histiocytosis
ankylosing spondylitis
allergic bronchopulmonary aspergillosis
radiation
tuberculosis

91
Q

What conditions affect lower lobes?

A

rheumatoid arthritis
asbestosis
scleroderma
cryptogenic fibrosing alveolitis
other (drugs, e.g. busulphan, bleomycin, nitrofurantoin, hydralazine, methotrexate, amiodarone)

92
Q

What are the features of primary ciliary dyskinesia

A

Absence of dynein arms
Abnormal real time electron microscopy study of nasal biopsy
wrong
Agenesis of frontal sinuses

93
Q

Is traction bronchiectasis an acute or chronic finding?

A

Chronic

94
Q

How do you treat UIP?

A
  • pirfenidome
  • nintedinib
  • lung transplant
  • pulmonary rehab improves QoL
  • mortality 2.5 years
95
Q

How do you treat cryptogenic organising pneumonia?

A

Corticosteroids - 0.5mg/kg
Good prognosis
Ass antisynthetase syndrome

96
Q

How do you treat hypersensitivity pneumonitis?

A

Remove trigger
Steroids

97
Q

Pulmonary lymphangioleiomyomatosis: PLAM
is associated with what?

A

Tuberous sclerosis complex
Treat PLAM with mTOR (Sirolimus)

98
Q

What are the cut offs required to get antifibrotics?

A

IPF diagnosis
FVC >=50%
FER >0.7
dlco >= 30%
No other cause identified

99
Q

What is associated with NSIP?

A

Connective tissue disease, HUV drugs - amiodarone, methotrexate, flecanide, nitrofurantoin - hypersensitivity pneumonitis

100
Q

How do you treat NSIP?

A

Glucocorticoids, second agents nycophenolaye, azathioprine, third line cyclophosphamide

101
Q

How do you diagnose sarcoidosis?

A

Bronchoscopy - lavage - elevated CD4:CD8
Biopsy - endobronchial or transbronchial,
EBUS - positive in 80-90%
HRCT- bilateral hilar and mediastinal lymphadenopathy

102
Q

What are indications for treatment of sarcoidosis

A

Progressive symptomatic pulmonary disease
Progressive loss of lung function
Cardiac disease
Neurological disease
Eye disease not responding to topical therapy
Symptomatic hypercalcaemia
Progressive extra-pulmonary disease

103
Q

How do you treat sarcoidosis?

A

ICS
PO steroids
MTX, hydroxychloroquine
AZA

104
Q

What defines a significant change in lung function in IPF?

A

FVC reduction by 10%, DLCO reduction by 15%
FVC is the greatest marker of mortality

105
Q

BAL with high lymphocytic count is indicative of what kind of interstitial lung disease?

A

Sarcoidosis, NSIP, Hypersensitivity, drug induced ( NOT IPF)

106
Q

BAL with hight neutrophil count indicates what kind of ILD?

A

IPF, asbestosis, infection

107
Q

What are the causes of UIP findings on HRCT?

A

IPF, RA related ILD, Asbestosis

108
Q

What obstructive diseases have decreased DLCO on spirometry?

A

Emphysema
Alpha-1 antitrypsin deficiency
Cystic fibrosi

109
Q

What restrictive diseases have decreased DLCO?

A

Interstitial lung disease or pneumonitis
With mixed picture
Sarcoidosis
Asbestosis
Miliary TB
Heart failure

110
Q

What Nomal spirometry conditions have decreased DLCO?

A

Pulmonary vascular disease (PE, pulmonary HT, Scleroderma)
Early interstitial lung disease
Anaemia - usually corrected for
Increased carboxyhemoglobin level: smoking (higher HbCO so higher gradient to diffuse against)

111
Q

What is the mutation required to get ivacaftor?

A

G55D1 Class III defect

FRACP Exam (15 decks)

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