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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
What is bronchiectasis?
Permanent dilation of bronchi and bronchioles due to destruction of airway muscles and elastic connective tissue
26
What are the immune effects of macrolides in chronic airway inflammation?
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)
27
What level is diagnostic for a chloride test?
>60mmol/l Borderline 40-60mmol/l - these need to go on to CFTR genotype testing
28
What does Ivacaftor do?
CFTR potentiator - opens the channel of the protein
29
What does tezacaftor/Elexacaftor do?
CFTR corrector - moves the protein to the cell surface
30
What is distal intestinal obstruction syndrome?
A complication of CF - impaction of secretions in the ileocaecal junction RIF faecal mass can mimic appendicitis Faeces in small bowel on AXR
31
What type of airway inflammation predominates in bronchiectasis?
Neutrophilic inflammation, occasionally eosinophilic
32
What are patients with primary immunodeficiency more at risk of in bronchiectasis?
Greater decline in lung function ( XLA, CVID) Treat with IVIG which would slow decline in FEV
33
What is bronchiectasis overlap syndrome?
When patients have bronchiectasis in association with another connective tissue disorder eg RA - they have worse outcomes
34
What are risk factors for having a bronchiectasis exacerbation ?
IgG2 deficiency, chronic bacterial infection, resp viral infections, hs exacerbation
35
What is Ivacaftor monotherapy not useful in?
F508del mutation
36
What are the dianostic criteria for ABPA?
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
37
What are the radiological findings of ABPA?
- 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%
38
What is the treatment for ABPA exacerbation?
Pred 0.5mg/kg weaned over 3 months to supress the immune response to aspergillis Long term treatment does not prevent relapse
39
How do you treat steroid dependent ABPA?
Anti-fungal Itraconazole/voriconazole Improved IgE, radiology and QoL and symptoms. Used for 16 weeks in patients who are steroid dependent.
40
What are the risks of use of itraconazole in APBA?
Itraconazole inhibits CYP3A4 increasing steroid concentration if on ICS - risk of cushings
41
CT findings of Nodules and cysts in the upper lung zones with recurrent pneumothorax is associated with what?
Pulmonary Langerhans cell histiocytosis
42
Pancoast tumours occur most commonly in what lung ca?
Squamous cell
43
What are the cut offs for mild, mod severe, very severe airflow obstruction?
Mild>70 Mod 60-69% Severe 35-49% Ver severe <35%
44
What do you do if spirometry suggests restrictive disease? (re4duced FVC, or reduced FEV1 and FCV)
Total lung volumes
45
What are spiro findings for fixed large airway obstruction ? (e.g tracheal stenosis)
Flat (plateau) expiratory loop AND inspiratory loop
46
What are spiro findings for intrathoracic variable obstruction?
Flat expiratory loop, with normal inspiratory loop. (INTRAthoracic effects EXPiratory loop)
47
Whats the spiro findings for an extrathoracic variable obstruction?
Normal expiratory loop and flat Inspiratory loop ( EXTRAthoracic, INSPiratory loop)
48
How do you calculate A-agradient at sea level ant normal temp?
A-a= (150-(1.25PaCO2))-PaO2)
49
How do you calculate H+ from a ABG?
H= 24(PaCO2/HCO3)
50
What is the ratio of HCO3 increasing to PCO2 increasing in acute and chronic respiratory acidosis?
Acute: HCO3 inc by 1mml/l for every 10mmHg PaCO2 Chronic HCO3 inc by 4mmol/L for every 10mmHg PaCO2
51
What are the radiological findings of UIP?
- Subpleural, basal predominantes - Honey combs - NOT ground glass - this is inflammatory Retucular abnormality
52
What are the radiologic features of Non-specific interstitial pneumonia
- Bilateral basal predominant ground glass opacification without honey combing or traction bronchiectasis
53
What are the radiologic findings of organising pneumonia?
Consolidation, bronchovascula in distribution
54
What are the radiologic findings of desquamative interstitial pneumonia
Ground glass with small cysts
55
What are the radiologic findings of hyprsensitivity pneumonitis acute vs chronic?
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
What adiologic pattern is associated with Idiopathic pulmonary fibrosis?
UIP (diagnosis requires exclusion of other causes of pulmonary fibrosis)
57
What is the mechanism of action of pirfenidone?
acts through TGF-B and reduces fibroblas proliferation
58
What is the MoA of nintedanib
Inhibits multiple TKIs (PDGF, VEGF, FGF)
59
What are the benefits of Pirfenidone and nintedinib?
Reduce rate of decline of FVC and improve survival
60
What are the SE of Pirfenidone?
Nausea and UGI side effects and photosensitivity rash
61
What are the side effects of nintedanib
Diarrhoea, weight loss
62
Features of lymphangioleiomyomatosis (LAM) ?
Combination of pulmonary cysts and renal angiomyolipoma in a young woman is a classic presentation
63
What is "Crazy paving" on CT chest? + what does it indicate
refers to the appearance of ground-glass opacities with superimposed interlobular septal thickening and intralobular septal thickening. Seen in Pulmonary alveolar proteinosis
64
What defines an apnoea and hypopnoea?
Apnoea - decresed sats and reduced air flow by 90% >10s Hypopnoea - Decreased airflow 30% and decresed sats by 3% >10s
65
What finding is different in OSA vs OHS?
Always have high pCO2 in OHS, not always in OSA
66
What are needed for dx OHS
Elevated pCO2 - during or immediately after sleep, BMI >35 and no other reason for pCO2
67
What is the treatment of central sleep apnoea?
No evidence of Bipap or Cpap, if treat patients with reduced ejection fraction there is increased mortality
68
How do you treat OHS?
CPAP with or without O2, BiPAP second line
69
How many overweight people have OHS?
15% people with BMI >30, 50% BMI >50
70
What is the mechanism of OHS?
Altered chemoresponsiveness, increased mechanical work, reduced lung volumes (ERV), and VQ mismatch.
71
What electrolyte is associated with restless leg?
Iron
72
What is narcolepsy?
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
What is cataplexy?
Sudden loss of motor power during the waekfulness due to REM intrusions
74
What are the types of nacolepsy?
Type 1 - narcolepsy with cataplexy Type 2 - Narcolepsy without cataplexy Acquired deficiency of orexin
75
How to treat narcolepsy?
T1 - Modafinil for narcolapsy, methylphenidate narc and cataplex
76
What are the 5 classes of pulmonary HTN
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
Whats the criteria for PAH?
mPAP >20mmHg PAWP <15mmHg PVR >2 WU
78
What is the most common cause of type 1 Pul HTN?
Schistosomiasis ( Idiopathic is second)
79
What gene is involved in hereditary PAH?
BMPR2 - bone morphogenetic protein AD with incomplete penetrance BMPR2 abnormalities result in proliferations of pulmonary vascular cells leading to vascular remodelling.
80
What are the 3 vascular mediators of PAH?
Incr Endothelin- Vasoconstricter and mitogen Decr NO - vasodilator and antiproliferative Decr Prostacyclin - Vasodilator, antiproliferative and inhibits platelet function
81
What do endothelin receptor A and B do?
Foundon smooth muscle, In combination cause vasocontriction Endothelin B alone on vascular endothelium, they release NO - antiproliferative effect
82
If patients are vasoreactive during a RHC, what med might they benefit from ?
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
What meds act on the endothelin pathway in PH?
Selective endothelin receptor antagonist - Ambrisentan (ETA) Dual endothelin receptor antagonight - ETA and ETB - Bosentan or macitentan
84
What meds work on NO pathway in PH?
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
What meds work on the prostacyclin pathway
Prostacyclin analogues - epoprotenol, treprostinil, iloprost Non-prostanoid IP receptor agonists - slecipag These increase cAMP leading to vasodilation
86
What things are in the ESC guidelines for risk assesment of PAH?
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
What is normal pulmonary arterial wegde pressure?
<12
88
What are the histological findings in PAH?
Intimal fibrosis, endothelial proliferation, medial thickening - smooth mucle cell hypertrophy and hyperplasia
89
What is CTEPH?
Chronic thromboembolic pulmonary disease: Mechanical obstruction of pul arteries by thrombus that doesnt resolve . SOB >3months anticoag
90
What conditions affect upper lobes?
Upper lobes (SCHART-S) silicosis (progressive massive fibrosis), sarcoidosis coal workers’ pneumoconiosis (progressive massive fibrosis) histiocytosis ankylosing spondylitis allergic bronchopulmonary aspergillosis radiation tuberculosis
91
What conditions affect lower lobes?
rheumatoid arthritis asbestosis scleroderma cryptogenic fibrosing alveolitis other (drugs, e.g. busulphan, bleomycin, nitrofurantoin, hydralazine, methotrexate, amiodarone)
92
What are the features of primary ciliary dyskinesia
Absence of dynein arms Abnormal real time electron microscopy study of nasal biopsy wrong Agenesis of frontal sinuses
93
Is traction bronchiectasis an acute or chronic finding?
Chronic
94
How do you treat UIP?
- pirfenidome - nintedinib - lung transplant - pulmonary rehab improves QoL - mortality 2.5 years
95
How do you treat cryptogenic organising pneumonia?
Corticosteroids - 0.5mg/kg Good prognosis Ass antisynthetase syndrome
96
How do you treat hypersensitivity pneumonitis?
Remove trigger Steroids
97
Pulmonary lymphangioleiomyomatosis: PLAM is associated with what?
Tuberous sclerosis complex Treat PLAM with mTOR (Sirolimus)
98
What are the cut offs required to get antifibrotics?
IPF diagnosis FVC >=50% FER >0.7 dlco >= 30% No other cause identified
99
What is associated with NSIP?
Connective tissue disease, HUV drugs - amiodarone, methotrexate, flecanide, nitrofurantoin - hypersensitivity pneumonitis
100
How do you treat NSIP?
Glucocorticoids, second agents nycophenolaye, azathioprine, third line cyclophosphamide
101
How do you diagnose sarcoidosis?
Bronchoscopy - lavage - elevated CD4:CD8 Biopsy - endobronchial or transbronchial, EBUS - positive in 80-90% HRCT- bilateral hilar and mediastinal lymphadenopathy
102
What are indications for treatment of sarcoidosis
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
How do you treat sarcoidosis?
ICS PO steroids MTX, hydroxychloroquine AZA
104
What defines a significant change in lung function in IPF?
FVC reduction by 10%, DLCO reduction by 15% FVC is the greatest marker of mortality
105
BAL with high lymphocytic count is indicative of what kind of interstitial lung disease?
Sarcoidosis, NSIP, Hypersensitivity, drug induced ( NOT IPF)
106
BAL with hight neutrophil count indicates what kind of ILD?
IPF, asbestosis, infection
107
What are the causes of UIP findings on HRCT?
IPF, RA related ILD, Asbestosis
108
What obstructive diseases have decreased DLCO on spirometry?
Emphysema Alpha-1 antitrypsin deficiency Cystic fibrosi
109
What restrictive diseases have decreased DLCO?
Interstitial lung disease or pneumonitis With mixed picture Sarcoidosis Asbestosis Miliary TB Heart failure
110
What Nomal spirometry conditions have decreased DLCO?
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
What is the mutation required to get ivacaftor?
G55D1 Class III defect

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