Respiratory Flashcards
ECG changes in PH
RAD
Tall P in II
Tall R in V1
poor R wave progression
Inferior TWI
RBBB
Asbestos Related Pleuropulmonary Disease
- Asbestosis
- Pleural disease
a. Benign asbestos effusion
b. Focal and diffuse benign pleural plaques - Malignancies
a. NSCLC
b. Small cell carcinoma
c. Mesothelioma
Asbestos Related Malignancy
Asbestos exposure RR of lung cancer of 3.5
- Compared with non-smokers without asbestos exposure,
- OR 1.70 (95% CI 1.31–2.21) among asbestos-exposed non-smokers,
- OR 5.65 (95% CI 3.38–9.42) among smokers without asbestos exposure,
- OR 8.70 (95% CI 5.8–13.10) among asbestos-exposed smokers
Chronic beryllium disease
Clinical featurs and CT findings
Beryllium is used in metal and alloy machine shops, electronics, ceramics, aerospace industries
Clincal features:
- cough and SOB (common)
- fever, night sweats, fatigue
HRCT findings:
- nodules in varying sizes
- thickened septal lines
- ground glass opacities
- cystic cavitation
- bronchial wall thickening
- adenopathy involving the hiulum or mediastinum
ABPA diagnostic criteria
- Asthma or CF
- need both: a) positive skin prick test or increased IgE levels to A. fumigatus b) Elevated IgE conc (>1000 IU)
- 2/3 of following:
a. Positive Aspergillus precipitants or IgG to A. fumigatus
b. Radiology consistent with ABPA
c. Total Eosinophil count >0.5 x 10^6 ub steroid naive pt
ABPA radiology findings
- 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%
Positive prognostic factors for Sarcoid
<40years
Asymptomatic LN on chest imaging
Acute inflammatory manifestation
Lack of evidence of organ failure or progression
Negativeprognostic factors for Sarcoid
- Age>40
- Pulm fibrosis on CT
- Lung function impairment
- Pulm. HTN
- Extrapulmonary involvement
- Lupus pernio
- Chronic hypercalcaemia
- Cystic bone lesions
CF mutation types:
Type 1: no functional CFTR
Type 2: F508del defect trafficking - most common
Type 3: G551D defect channel opening
Type 4: defect in conduction
Type 5: low quantity
Microscopic changes in lung with ageing
recoil, collagen, aveoli, compliance
Old:
reduced recoil
increased compliance
reduced aveoli volume
reduced collagen1 and increased collagen 3
Factors associated with an increased incidence of HAPE
Male
cold ambient temperatures
preexisting respiratory infection
vigorous exertion
Preexisting conditions or anatomic abnormalities that lead to increased pulmonary blood flow, pulmonary hypertension, or increased pulmonary vascular reactivity may predispose to HAPE, even at altitudes below 2500 m. These include:
1. primary pulmonary hypertension
2. congenital absence of one pulmonary artery
3. left-to-right intracardiac shunts, such as atrial septal defects and ventricular septal defects.
Describe Haemophilus influenzae on a gram stain
Gram -ve bacilli
Describe Moraxella catarrhalis on a gram stain
Gram negative diplicoccus
Which lung nodule does not need FU?
solid nodules 6 mm or less in diameter in low-risk adults >35 years old generally need no further follow-up.
Low glucose in pleural fluid?
Malignancy
TB
SLE
Oesophageal rupture
RA
Mechanism of isoniazid resistance in TB?
Isoniazid acts by inhibiting the synthesis of mycolic acids through the NADH-dependent enoyl-acyl carrier protein (ACP)-reductase. This drug requires the activity of mycobacterium catalase peroxidase to be activated, therefore mutations in the enzyme lead to drug resistance.
What causes rifampicin resistance in TB?
Mutation in RNA polymerase
What is the RAPID score?
looks at the individual factors in infected pleural effusion that confers an adverse prognosis
- Advanced age
- absence of purulent fluid
- low albumin
- increased serum urea
- presence of hospital acquired infection
Mycophenolate is not used routinely as studies have shown inferiority compared to AZA- which condition?
GPA
Direct vs indirect provocation tests
Direct: methacholine, histamine
- sensitive, not specific
- use bronchodilators
Indirect: hypertonic saline, mannitol, adenosine
- specific for inflammation, thus asthma
- use ICS
COPD triple therapy
LAMA/LABA/ICS
reduced mod-severe exacerbation
reduced hospitalisation
better lung function
BUT, increased pneumonia (even cf LABA/ICS)
CI for spirometry
- High: recent MI, PE, AAA, pneuomothorax
Acute illness (relative)
SBP >200 or DBP >120 - Mod: major thoracic, abdominal, head surgery
- Less serious surgical procedures
RF for group 1 PAH
CTD: SSc, Raynaud’s SLE, MCTD, RA
HIV
Portal HTN
CHD: septal defect, Eisenmenger syndrome
Schistosomiasis (most common worldwide)
Drugs:
- Dasatinib
- Toxic rapeseed oil
- Methampetamines
Possible:
- Leflunamide
- Alkylating agents
- IF alpha/beta
- Amphetamines
- St John’s wort
- L-tryptophan
- Cocaine
BMPR2 mutation
- accounts for 70-80% of familial PAH (type1)
but only 25% with BMPR2 mutation develop PAH - Tend to present early, more severe dx, increased risk of death
AD with incomplete penetrance
Other genes: ALK1, ACVRL1, 5HTT, ENG, SMAD9, KCNK3, CAV 1
RF for CTEPH
pro-coagulant state
Lupus anticoagulant
APS
Splectomy
indwelling IV line
Permenant IV devices
IBD
PCRV and ET
Malignancy
**High dose thyroid replacement **
PVOD
What is selxipag?
Primary ciliary dyskinesia
Agenesis of frontal sinues
absent dynein arm
Abnormal real time electron microscopy study of nasal biopsy
Narcolepsy and HLA
HLA DR2
Can PJP be culutred?
Pneumocystis cannot be cultured, the diagnosis relies upon the visualization of the cystic or trophic forms in appropriate specimens. Stains that have commonly been used selectively stain the cell wall of the cystic form, and include **Gomori-methenamine silver **
What’s the first line treatement of cataplexy?
Venlafaxine
Treatment of narcolepsy without cataplexy?
Modafinil is first line for the somnolence associated with narcolepsy
Pregnancy and lung function?
- The forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC ratio, and peak expiratory flow do not change significantly during normal pregnancy.
- Residual volume** (RV) and functional residual capacity (FRC)** decrease during pregnancy, while total lung capacity (TLC) decreases only slightly in the last trimester
- Minute ventilation increases during pregnancy, associated with increased tidal volume, presumably due to increased circulating levels of progesterone. Thus, normal pregnancy is associated with a **compensated respiratory alkalosis. **
Change in sleep with age:
- total sleep time decreases
- sleep onset or latency becomes delayed
- there is an increase in daytime napping
- increase in awakenings and arousals
- decreased sleep efficiency
**- increased stage 1 and 2 sleep - decreased stage 3 and 4 (slow wave sleep)
- decrease in REM sleep **
- there is fewer sleep cycles at night. The circadian phase is also typically advanced (ie early to bed and early to rise)
VTE risk with pregnancy
The risk of VTE is increased equally across all three trimesters, however the risk is highest post partum (with VTE risk increasing 4x)
HAPE
HACE
LENT score
when do you use it?
what’s in it?
Riociguat
Stimulator of soluble guanylate cyclase indicated for the management of persistent or recurrent chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension.
CI to lung cancer surgery
SVC obstruction, FEV < 1.5, MALIGNANT pleural effusion, and vocal cord paralysis
What are typical CT findings for UIP?
Honey combing
Traction bronchiectasis
Lower lobe and peripheral predominance
No atypical features
Features that suggest UIP is unlikely
Subpleural sparing
Peribrochovascular predominance
Groundglass separate from honeycombing
Mosaic attenuation
Cysts
Diffuse nodules
Consolidation
RF for IPF
Age 50-70
Male
past smoking history
no alternative cause
Tell me about PALM
It’s sporadic cystic lung disease
associated with tuberous sclerosis complex
on CT you can see DIFFUSE bilateral cystic disease
FEMALES only
Associated with angiomyolipomas in the kidneys and meningiomas
complications: chylothorax, pneumothorax
Tx:
- Lung transplant
- Sirolimus
LIP
LCHC
What causes plateuing of inspiratory loop but not expiratory (variable extrathoracic obstruction):
Vocal cord paralysis - unilateral
In a flow vol loop
Normal inspiration: variable intrathoracic problem - trachiomalacia
Normal expirtion: variable extrathoracic problem
Both affected: fixed
Low DLCO, but high KCO
think loss of lung:
- prev. lobectomy
- severe pleural disease
- Kyphoscoliosis
- Diaphragmantic paralysis
Increased DLCO
SAMPLE:
- severe obesity
- asthma
- Mild LVF
- POlycythaemia
- Pulm. haemorrhage
- L->R intracardiac shunt
- Exercise
which drug to avoid in narcolepsy
Prazosin
serum neuron specific enolase: which cancer is it associated with
SCLC
Hounsfield units; when is it used
Nodules - >164 is likely to be benign
poor prognostic factors in CF
Low FEV1, High CO2, High WCC, Female
Elexacaftor/Ivacaftor/Tezacaftor - are beneficial in which phenotype?
Phe508del - gating or residual function
What is the most common symptom in bronchiectasis>
productive cough
CT features of bronchiectasis:
Bronchoarterial ratio>1
Lack of airway tapering
Airway visibility within 1cm of the costal pleural margin
Touching the mediastinal pleura
Indirect signs:
-bronchial wall thickening
-Mucus impaction
-Mosaic perfusion/air trapping on exp. CT
What is a signet ring sign on CT-chest?
Associated with bronchiectasis - where the bronchi is much larger than the associated blood vessel
Common causes of bronchiectasis:
- Idiopathic (40%)
- Post infectious (30%)
- Immunodef
- COPD
- CTD
- ABPA
- PCD
- Asthma
- NT mycobacteria
Lung function and primary immunodeficiency?
Primary immunodeficiency is associated with worseing lung fucntion - esp XLA
High dose IVIG has shown to be helpful in reducing the rate of decline
Bronchiectasis overlap sx
BE-RA
BE-COPD
worse outcome
Bronchiectasis severity score
Age, BMI, FEV1
Hospital admission, # of exacerbation
MRC score
P.aeruginosa
Other organism
CT severity
Which Ig def is associated with increased exacerbation in BE?
IgG2
Macrolide for exacerbation prevention
Azithromycin
- Both immunomodulatory effect and antibacterial effect
- Azithromycin 500mg 3 x week showed reduction in frequency of exacerbation
- But, no difference in:
- FEV1, QoL (but longterm macrolide use improved QoL), colonisation, sx
- greater benefit in pseudomonas colonisers
The effect of pseudomonas in BE
- worse mortality and morbidity
CF diagnostic criteria
- 1 or more:
-classic phenotype
- sibling with CF
- positive newborn screen
2.>1:
- abnormal sweat test on 2 or more occasions
- 2 CFTR variants identified
- abnormal nasal potential differential testing
What’s the chloride level on sweat test signficant for CF?
> 60 = highly likely
<29 = unlikely
What are the 2 most common clinical manifestation of CF?
Sinusitis
MAle infertility
Pseudomonas and CF?
70% of CF adults are chronically colonised
Mucoid phenotype is associated with worse lung fucntion
Azithromycin in CF
- Improves FEV1
- Reduced exacerbation
- -antibacterial effcet against pseudomonas
- anti inflammatory effect
- reduces biofilm production
DO NOT USED in NT mycobacterium colonised pt as increased risk of resistance
Airway secretion management in CF?
inhaled Dornase alpha:
- reduced exac
- improved FEV1
- improves QoL
inhaled hypertonic saline
- reduces exac
PEP:
- reduces exac
Most frequent pathogen in CF
- Staph aereus ealier in life
- then pseudomonas after 35
Haemophilus is higher in early life subsides after teenage
Burkholderia cepacia complex in CF
- Chronic infection results in **accelerated decline **in lung function and
shortened survival - Usually, multi-drug resistant
* Worse outcomes with lung transplantation esp with B. cenocepacia - Infection control measures to prevent cross infection between CF patients
*Non-tuberculous mycobacteria in CF: 10-20%
MAC is not assocaited with worse transplant outcome
M. abscessus IS associated with worse lung fucntion and transplant outcome
Ivacaftor and G551D mutation
G551D mutation is assocaited with Type 3 CF
- defect in channel opening
- Ivacaftor has shown to improve FEV1, reduced exac risk, reduce hospitalisation
- resutls in weight gain (which is good for CF pts)
Lumacaftor and Ivacaftor in DeltaF508
Detla F508 is the most common mutation in CF
Type 2 CF - problem with misfolding od protein and trafficking to mb
Lumacaftor partially corrects the misfolding and Ivacaftor helps with channel opening
Small improvement in FEV1, QoL and BMI
Reduced exac risk
but ~15% don’t tolerate it
In heterozygote the only improvement was int he sx score and sweat chloride level
Tezacaftor/Ivacaftor data
In F508 homozygotes:
- improved QoL, exac, FEV1 but not change BMI
in F508 hetero:
- the combo showed improvedment in FEV1 and QoL
- cf. ivacaftor monotherapy and placebo
Elexacaftor/Teza/Ivacaftor
in delta F508:
Heterozygotes: improved cl transport to ~50% of normal
Improved FEV1 and reduced exac
Homozygotes: improved it to >50%
improved QoL
In homozygotes:
Triple therapy cf Teza/Iva: improved FEV1 by 10% and QoL
Triple therapy in F508 mutation:
- improved FEV1 by ~15%
- Weight gain
- reduced LTOT, noctural NIV need, PEG feeding, transplant
Positional OSA is more prevalent in young and non-obese pts
REM OSA is more common in female and is associated with HTN, CVD, neurocognitive sx, insulin resistance
OSA with COPD –> increased risk of death and exacerbations
In HF, OSA or CSA increases mortality risk
In diabetes, CPAP improves glycaemic control
OSA is common in non-dialysis CKD
OSA and hypoxia may predispose to NASH in severe obesity
Increasing OSA severity is associated with increased risk of HTN
Benefit of wt loss in OSA
10% weight loss –> 25% improvement in OSA
Does CPAP have an effect on HbA1c
no
Proven benefit of CPAP
Reduced AHI
Improves symptoms
Reduced MVA
Improves erectile dysfunction
Improves QoL
Improves insulin resistance in non-diabetics with OSA
Potential improvement in HTN; more beneficial in more severe OSA with high baseline BP or resistant HTN
OSA and CVD evidence
- reduced risk of revascularisation after PTCA in CPAP treated OSA pt
- CPAP modestly lowers BP by 2-4mmHg in pts with mod-severe OSA
- CPAP did not reduce cardiovascular events in secondary prevention trial in mod-severe OSA
- Retrospective study
- There was an observational study that showed benefit with CPAP
Oral appliances and OSA
reduces AHI
useful in all severity but less responsive cf. CPAP in mod-severe OSA
reduction in AHI with CPAP is greater, but OA has a better compliance
improves QoL
Risk factors for CSA and CSR in HF
Male, >60
AF, low LVEF, daytime hypocapnia PaCO2<38
RLS is associated with
Willis-Ekbom sx
iron deficiency
Coeliac
ADHD
COPD
Depression/Panic
Fribromyalgia
PD
MS
Migraines
Drugs:
Dopamine antagonist
SSRIs
Neuroleptics
Lithium
Beta blockers
Diagnostic criteria for narcolepsy
MSLT<8mins or sleep latency (on PSG) <10min, REM sleep latency <20min
AND:
>2 sleep onset REM
REM within 15mins on PSG is suggestive of narcolepsy
REM behavioural disorder
-associated with synucleinopathies (LBD, PD, MSA)
- very common with PD
parkinsonism improves during RBD
- can predate dementia by 10years
- lack of REM atonia on PSG
Tx:
- clonazepam
- melatonin
Somnambulism
Sleep walking
peak incidence 11-12 years
Associated with HLA DQB1
commonly arises in nREM
RF: sleep dep, emotional distress, febrile illness, physical activity
Klein-Levin syndrome
Periodic hypersomnia
HLA association to narcolepsy
HLA DQB1*0602
DR3
What type of hypersensitivity reaction does ABPA illustrate?
Type 1,3, 4b
Which ILD resolves with smoking cessation?
RBILD and DIP
