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MRCP Part 2 and PACES > Respiratory > Flashcards

Flashcards in Respiratory Deck (89)
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1
Q

Sarcoidosis.

A

Typical presentation : chronic cough. fatigue. lethargy, erythema nodosum (Lofgren’s syndrome Hilar / EN / Arthropathy), Lupus Pernio

Atypical presentation: 5% neurological complications. optic neuritis. mononeuritis multiplex. myelopathy. seizures. granulomas causing mass effect. Basal meningitis with cranial nerve palsies. bulbar palsy. neuropsychiatric manifestations

2
Q

Causes of clubbing

A

NOT COPD

ABCDEF
abscess + asbestosis
bronchiectasis
CF
Dirty tumours - SCLC / mesothelioma / bronchogenic
Empyema
Fibrosing alveolitis / aspergillosis - any pulm fibrosis

CLUBBING AND CRACKLES = FAB
fibrosing alveolitis
Asbestosis
Bronchiectasis / bronchogenic cancers

OTHERS:
sarcoid / TB
RA
thyroid acropatchy

think cardiac / abdomen / thyroid

4 grades

1) loss of nail bed angle - SCHAMROTHs sign
2) boggy nail bed
3) Increased nail curvature
4) drumsticks

3
Q

lung function tests

A

Include
Spirometry:
FVC = from peak of inspiration forced expiration - exhale for as long and as forcefully as possible
FEV1 = Lung volume expelled in 1 second
FEV1/FVC
<70% = obstructive = i.e obstruction to expiration
Tumour / COPD / asthma / pulmonary fibrosis
>70% = restrictive
trapped lung / lobectomy / pulmonary fibrosis / flail chest

Diffusion capacity:
Mixture of CO and helium inhaled
CO crosses alveolar membrane
helium does not
percentage of CO that is transferred across is calculated = TLCO - total lung carbon monoxide
Total lung volume is then calculated using remaining helium concentration which would be diluted by gas already present in lung on inspiration
gives KCO = gas transfer occuring per unit volume based on CO loss and helium remaining

low kCO - low transfer - emphysema / ILD
high kCO - high transfer where blood++ present - PE / SLE / Wegners / anti GBM

Total lung capacity - TLC
Measured using helium dilution again as above
i.e. increased dilution of helium means more air present or trapped at beginning of inspiraiton

Increased in COPD / asthma / emphysema / pulmonary fibrosis / hyperinflation

Decreased in restrictive lung conditions

Flow volume loops
Expiration - above x axis
inspiration - below y axis
measures TLC and gives a plotted course of air flow

1)normal flow loop
2)obstructive flow loop - expiration sharp decrease and then soft plateau to 0 due to air trapping
3)restrictive - sharp insp and exp peaks and trough with narrowed base to loop diagram as less volume moved and more rapid transition of gas in an out
4) fixed obstruction extra thoracic
shallow inspiration - stridor
normal exp loop but smaller as paints a restrictive picture - expiration clears obstruction
5)fixed upper air way stenosis - tumour / trauma
shallow inspiration and expiration flow loops
6)varibale upper airway obstruction
obstruction during expiration only so shallow expiration but sharp inspiration - i.e. inspiration clears obstruction

4
Q

What is diffusion capacity

A

Essentially a measure of gas transfer and may indicate underlying pathology

Diffusion capacity:
Mixture of CO and helium inhaled
CO crosses alveolar membrane
helium does not
percentage of CO that is transferred across is calculated = TLCO - total lung carbon monoxide
Total lung volume is then calculated using remaining helium concentration which would be diluted by gas already present in lung on inspiration
gives KCO = gas transfer occuring per unit volume based on CO loss and helium remaining

5
Q

What is total lung capacity and how is it measured

A

TLC is the measured total useable lung volume.
It is measured using helium and carbon monoxide.
CO crosses the lung alv membrane
Helium does not
The concentration of helium is measured on expiration as it will dilute proportionately according to the concentration of gas present at the beginning and end of inspiration

Therefore TLCO will be increased in any resp condition where air trapping or hyperinflation are present

TLCO will be reduced in any condition where lung volume is restricted

6
Q

explain flow volume loops

A

Flow volume loops
Expiration - above x axis
inspiration - below y axis
measures TLC and gives a plotted course of air flow

1)normal flow loop
2)obstructive flow loop - expiration sharp decrease and then soft plateau to 0 due to air trapping
3)restrictive - sharp insp and exp peaks and trough with narrowed base to loop diagram as less volume moved and more rapid transition of gas in an out
4) fixed obstruction extra thoracic
shallow inspiration - stridor
normal exp loop but smaller as paints a restrictive picture - expiration clears obstruction
5)fixed upper air way stenosis - tumour / trauma
shallow inspiration and expiration flow loops
6)varibale upper airway obstruction
obstruction during expiration only so shallow expiration but sharp inspiration - i.e. inspiration clears obstruction

7
Q

What is COPD

A

Progressive airflow obstruction which demonstrates very limited reversibility with bronchodilators

Associated with various pathologies but commonly due to a hyper responsiveness to inhaled irritants like cigarette smoke / silica / coal dust / specific allergen / other particulates

2 types
1. Chronic bronchitis - chronic productive cough for at least 3 months of the year over 2 consecutive years

  1. Emphysema: defined histologically or via CT demonstrating
    a) alveolar wall destruction leading to permanent and fixed enlargement of air spaces distal to end bronchioles
    b) bronchiectasis
8
Q

What is emphysema

A

Emphysema is a form of COPD defined histologically or radiologically as the destruction alveoli walls leading to permanent and fixed enlargement of the airways distal to the end bronchioles.

9
Q

What is bronchitis

A

Bronchitis is defined as a chronic productive cough lasting for 3 months successively over 2 years. It is a form of early COPD

10
Q

Signs of COPD

A

pursed lips breathing - self peep to prevent airway collapse
cachexia - increased RR / catabolic process / malignancy
accessory muscle use
reduced cricosternal distance - <3cm
prolonged expiratory phase
expiratory wheeze
crackles - bronchiectasis
expiratory crackles - large airway collapse
barrel chest - hyperinflation
asterixis - CO2 retention flap
tar stained fingers
Hyper resonance to percussion - reduced hepatic and cardiac dullness -HYPERINFLATION
Reduced breath sounds over bullae
Focus of wheeze - infection - apex ?TB
day time somnolence CO2 retention / OSA

11
Q

Grade COPD

A
  1. MRC Dyspnoea scale
  2. FEV1 Gold scale
  3. BODE index
Dyspnoea scale
1 - not troubled
2- SOB hurrying flat or slight incline
3 - own pace due to SOB or stops for breath at own pace
4- limited to 100m 
5-house bound or SOB dressing

Gold Scale where FEV1/FVC <70%

  1. Mild FEV1>80%
  2. moderate FEV1 50-80%
  3. severe FEV1 <50%
  4. very severe FEV1 <30% OR <50% + T2RF
BODE
BMI
FEV1
6 Min walk test
mrc dyspnoea scale
12
Q

What is the BODE index

A

BODE index is a predictor of COPD outcome- admissions and mortality - which takes into account pulmnary and extrapulmonary manifestations of COPD

BMI
Obstructive manifestations - FEV1 (GOLD)
Dyspnoea (MRC)
Exercise tolerance - 6 min walk test

score = 0-10
weight loss is a predictor of poor outcome and decline

13
Q

How to investigate / diagnose COPD

A
1. Pulmonary function tests
Flow loop
LIMITED REVERSIBILITY <15% CHANGE IN FEV1
FEV1/FVC <70%
FEV1 - gold grading - BODE
6 minute walk test - BODE
BMI - BODE
MRC Dyspnoea scale - BODE
2. Signs and symptoms
BRONCHITIS - CHRONIC SUPPURATIVE COUGH FOR >3 MONTHS IN  2 SUCCESSIVE YEARS
barrel chest
exp wheeze / prolonged exp phase / cricost <3cm
weight loss
SOBOE
Asterixis
3. CXR
4. CT - emphysema / bullae / bronchiectasis
5. Hb - polycythaemia
6. GAS - CO2 retention
7. alpha 1 antitrypsin  - EARLY ONSET COPD
8.sputum culture
9.throat swab / viral throat swab
14
Q

COPD exacerbation causes

A
60% viral induced exacerbations or bacterial
30% unknown
10% pollution
VIRAL: rhinovirus / adenovirus / influenza / coronavirus
BACTERIAL: Strep Pneumoniae
1. Moraxella Caterhallis
2. Haemophilus 
3. pneumococcal
4.mycobacterium - TB
15
Q

Smoking Cessation

A

Smoking Cessation improves symptoms and FEV1 decliine slowed and return to near normal for age after 5 years
ONLY TREATMENT SHOWN TO ALTER DISEASE COURSE

5As = BTS approved
or 
smoker? 
ready to stop? 
you should quit! 
Ill help! 
come back!
1. Ask about smoking status
2. Assess readiness to quit
3. advise to quit
4. assist in efforts to quit
5.Arrange follow-up

Therapy:
Identify quit day
group therapy / counselling
inform of abnormal lung funtion

Nicotine replacement therapy
limited evidence one form over another
better compliance with nasal inhaled NRT

also BUPROPRION - NORADRENALIN AND DOPAMINE REUPTAKE INHIBITOR

VARENICLINE
Partial agonist of NICOTINIC ACH RECEPTORS

16
Q

NICOTINE REPLACEMENT THERAPY

A

Limited evidence to suggest one form better than another

inhaled forms may cause bronchospasm

nasal NRT show better compliance anecdotally

BUPROPRION - NA AND DOPAMINE AGONIST

VARENICLINE - NICOTINIC RECEPTOR AGONIST

5As
assess smoking 
assess readiness to quite
advise to quit
advise on how
arrange follow up

GROUP COUNSELLING
NRT

17
Q

COPD TREATMENTS

A
ACUTE:
infection?
amox  / clarith
steroid 5 days and wean
pesuodomonas - Tazocin 10 days
GAS - retention C02 - ?NIV
Chronic
Mucolytics - glycopyronnium or carbocisteine
Early morning GAS - ?nocturnal NIV
sputum culture
chest physio
Tailor sats
Chronic T2RF - NIV or LTOT
smoking cessation - 5As / NRT / Buproprion / Varenicline
rescue pack
PULMONARY REHAB
expectoration training
psychological and CBT
nutritional advice
physical training
IMPROVES EX TOLERANCE / QOL / REDUCES ADMISSION

Optimise nutrition

Vaccination
pneumococcal >65 or <65 if FEV1 <40%
influenza

ladder:
STEP 1  
SABA - monotherapy
salbutamol
SABA + SAMA
salbutamol OR TURBUTALINE + atrovent (ipratropium)

STEP 2
LABA OR LAMA - monotherapy
Tiotropium or salmeterol / formeterol

TORCH study recommends Long acting if > 2 exacerbations per year

LABA + LAMA
TIOTROPIUM (UPLIFT study)+ SALMETEROL (TORCH) or Formeterol

STEP 3
LABA + ICS or LAMA + ICS (FEV1 <50% + >2 exacerbations per year)
SERETIDE / FOSTAIR / SYMBICORT + Tiotropium

STEP 4
BOTH LABA + ICS + LAMA + Theophyllin + pulm rehab
+ tiotropoium long acting anticholinergic (bronchodilation)
SERETIDE - salmeterol +fluticasone - steroid
FOSTAIR - formeterol + becolometasone
SYMBICORT = formeterol + budesonide

Add Roflumilast - PDE-4 antagonist where

  • already on triple therapy
  • Gold >3 FEV1<50%
  • > 2 exacerbations per year

Add Theophyllin
UNCLEAR MECH OF ACTION / RELAXES SMC / improves Mucociliary clearance
Lots of interactions
difficult therapeutics esp in obese

Step 5 - FEV1 <30% MRC 4-5
LTOT
LTOT ambultatory needs to be used >15 hours per day
NON SMOKER
Pa02 <7.3 + high Hb + noct T1RF / pulm htn / core pulm

IMPROVES SURVIVAL AND QOL

Step 6
NIV
improve QOL
reduces admissions
improves sleep quality and resp fatigeu
RESETS RESP CENTRE LEADING TO IMPROVED DAY TIME T2RF

+/- rescue pack
+ smoking cessation - 5As + NRT + buproprion / varenicline
+ nutrition
+ vaccination

18
Q

COPD Treatment ladder = 3 steps - describe + additional therapies

A
ladder:
STEP 1  
SABA - monotherapy
salbutamol
SABA + SAMA
salbutamol OR TURBUTALINE + atrovent (ipratropium)

STEP 2
LABA OR LAMA - monotherapy
Tiotropium or salmeterol / formeterol

TORCH study recommends Long acting if > 2 exacerbations per year

LABA + LAMA
TIOTROPIUM (UPLIFT study)+ SALMETEROL (TORCH) or Formeterol

STEP 3
LABA + ICS or LAMA + ICS (FEV1 <50% + >2 exacerbations per year)
SERETIDE / FOSTAIR / SYMBICORT + Tiotropium

STEP 4
BOTH LABA + ICS + LAMA + Theophyllin + pulm rehab
+ tiotropoium long acting anticholinergic (bronchodilation)
SERETIDE - salmeterol +fluticasone - steroid
FOSTAIR - formeterol + becolometasone
SYMBICORT = formeterol + budesonide

Add Theophyllin
UNCLEAR MECH OF ACTION / RELAXES SMC / improves Mucociliary clearance
Lots of interactions
difficult therapeutics esp in obese

Step 5 - FEV1 <30% MRC 4-5
LTOT
LTOT ambultatory needs to be used >15 hours per day
NON SMOKER
Pa02 <7.3 + high Hb + noct T1RF / pulm htn / core pulm

IMPROVES SURVIVAL AND QOL

Step 6
NIV
improve QOL
reduces admissions
improves sleep quality and resp fatigeu
RESETS RESP CENTRE LEADING TO IMPROVED DAY TIME T2RF
\+/- rescue pack
\+ smoking cessation - 5As + NRT + buproprion / varenicline
\+ pulm rehab
\+ nutrition
\+ vaccination
19
Q

SURGICAL TREATMENT COPD

A

LUNG REDUCTION
BULLECTOMY
TRANSPLANT

  1. LUNG REDUCTION THERAPY
    excision of non functional lung allows for inflation of trapped / physiologically normal lung
    REDUCES MORTALITY
    IMPROVES QOL

REQUIREMENTS:
TLCO >20% (diffusion capacity demonstrating functional lung tissue
FEV1 >20%
paC02 <7.3 i.e minimal evidence of T2RF

2. Bullectomy
excision of large bullae
reduces risk of PTx
restores elastic recoil
reduces air resistance and air trapping
increases functional residual capacity

REQUIREMENT:
PROGRESSIVE DYSPNOEA
FEV1<50% - GRADE 3 GOLD
BULLAE >1/3 HEMITHORIAX WITH PRESERVED HISTOLOGY SURROUNDING

  1. Lung Transplant
    IMPROVES FUNCTIONAL CAPACITY
    1 year survival = 78%
    5 year 51%

REQUIREMENT:
BODE >5

20
Q

CPAP - HOW DOES IT WORK

A

Continuous positive pressure airway ventilation

Recruites alveoli that have collapsed and therefore improves airway compliance
splints open upper airway
forces fluid back into pulmonary vasculature
improves oxygenation

21
Q

Pneumonia Signs / symptoms

A

Pneumonia:
X ray evidence of collapse / consolidation within the lung parenchyma

  1. productive / supporative cough - new
  2. RR + pyrexia
  3. tracheal tug . IC recession. SC recession
  4. T1Rf
  5. pleuritic CP
  6. haemoptysis
  7. Increased diffusion gradient in prescence of pulmonary haemorrhage otherwise reduced
  8. focal crackles with dullness to percussion with increased vocal phremitis over it ?effusion
  9. clubbing if chronic / SCLC / bronchiectasis
  10. effusion / empyema - acidotic septated consolidative effusion
  11. bronchial breathing
  12. dysphagic ?MND / GBS / stroke - ASPIRATION

Erythema multiforme - target lesions - MYCOPLASMA
Erythema nodosum - nodes of erythema not dissimilar to jane way lesions (different aetiology)
bullous myringitis - lesions on tymp membrane
cold haemolytic anaemia - cold agglutinins /raised ldh / splenomeg if assoc spherocytosis

22
Q

Pneumonia severity

A

CURB65 SCALE CORROLATES WITH 30 DAY MORTALITY

NEW CONFUSION
UREA >7
RR>30
BP <90

OVER 65

SCORE /5
0 = 0.7% - treat at home
1= 3% = home
2 = 10% = increased risk
3 = 17% = severe ITU
4 = 41% = severe ITU
5=57% = severe ITU
23
Q

pneumonia investigations

A
Bloods panel
ABG
eosinophilia?
CXR
USS if indicated by CXR and clinical
pleural tap if indicated +/- chest drain if empyema or resp compromise
throat swab  / cultures
atypical screen
pneumococall Ag
Mycoplasma- erythema nodosum / mulitforme +HAEMOLYSIS - cold agglutinins + raised LDH
legionella - travel
AFBs - travel / severe / CXR pulm TB or miliary / immunosup
HIV - severe presentation and young
flu swab

Ct chest
BAL

24
Q

pneumonia definition

A

an acute or chronic resp illness that is marked by inflammation of lung tissue accompanied by infiltration of alveoli and often bronchioles with white blood cells (such as neutrophils) and fibrinous exudate seen on CXR

25
Q

common causes of CAP

A

Common bacterial

  1. strep pn up to 40% - esp splenetomy
  2. haemophilus influenzae - mroe in copd
  3. mycoplasma - erythema multiforme / marginatum + HAEMOLYSIS + bullous myringitis
  4. Q fever - chlamydophila
  5. legionella
  6. pneumococcal
  7. staph
  8. moraxella catarrhalis
  9. gram negs - aspiration

Atypical
Mycoplasma
pneumococcal - liver dysfunction
chlamydia

C.psitacci
C. burnetti

Viral
influenzae A/B

26
Q

Treatment pneumonia

A
  1. Abx
    amox and clarith +/- gram neg cover in aspiration
  2. target sats and appropriate oxygen therapy
  3. pen Ax - teicoplanin and clarith / flouroquinolone

6 week CXR + FU
consider HIV screen based on presentation

Vaccination

27
Q

HAP definition

A

Hospital aquired pneumonia with Sx presenting >72 hours post admission

USually gram neg org / pseudomonas

28
Q

Define Bronchiectasis

A

Permanent splinting open and destruction of middle and large bronchioles due to

a) chronic infection or inflammation
b) poor drainage
c) airway obstruction (continued infection)
d) defective immune response

29
Q

Causes bronchiectasis

A

Many causes!

Surgical sieve

V: vascular
NONE

I: infective/inflammatory
CYSTIC FIBROSIS
FIBROSING PULMONARY ALVEOLITIS - ASPERGILLOUS
TB
HIV
T: traumatic / mechanical - BRONCHIAL OBSTRUCTION
COPD
GRANULOMA - SARCOID TB
CARCINOMA
FOREIGN BODY
LN COMPRESSION

A: autoimmune / OVERACTIVE / UNDERACTIVE
ALLERGIC ASPERGILLOSIS
A1 ANTITRYPSIN

HYPOGAMMAGLOBULINAEMIA
SCID
HIV

M: metabolic
RECURRENT ASPIRATION

I: iatrogenic/idiopathic

N: neoplastic
Bronchogenic cancer

C: CONGENITAL
CF
Ypungs Syndrome - CF without CFTR D508 - middle age
CP - aspiration
YELLOW NAIL SYNDROME - yellow nails - abn lymphatics - pleural effusion (chylothorax)
KARTAGENERS SYNDROME = PRIMARY CILIA DYSKINESIA: Dextrocardia / ciliary dyskinesia +bronchiectasis / otitis / subfertitily (similar to CF)
A1 Antitrypsin

30
Q

Surgical sieve

A
V: vascular
I: infective/inflammatory
T: traumatic
A: autoimmune
M: metabolic
I: iatrogenic/idiopathic
N: neoplastic
C : congenital
31
Q

Bronchiectasis imaging changes

A

CXR

CYSTIC BRONCHIECTASIS
Cystic sac distribution

CYLINDRICAL BRONCHIECTASIS
Tramlines / Ring shadowing

Severe - see honeycombing - lots of ring shadowing showing fibroses / inflammed

HRCT - high sensitivity and specificity
REID CLASSIFICATION
Cylindrical - most advanced
cystic
SIGNET RING SIGN - bronchi 1.5x thickness of adjacent artery =Op
Fluid levels in bronchi
Mucus plugs - bronchocoeles

OBLITERATIVE BRONCHIOLITIS - TREE IN BUD APPEARANCE

32
Q

Specific tests for bronchiectasis

A

HRCT - definitive
CF GENOTYPING / SWEAT TEST >60mmol CL - AUT REC
KARTAGENERS = primary ciliary dyskinesis - AUT REC
TB - Tuberculin skin test / Quantiferon

33
Q

Cystic fibrosis - What is the commonest mutation and pathophysiology of the disease process

A

CF transmembrane conductance regulator protein (CFTR)
Transporter channel which regulates chloride ICM and ECM balance.
Inhibits voltage gated Na channel from reabsorbing Na from ECM
Therefore regulates water movement across cell membrane and therefore ECM viscosity via regulation of NaCL.

Commonest mutation is DF508 - >90%
Autosomal recessive

When defective Chloride ions fail to be pumped back into cell
eNAc not inhibited and as ECM Na > ICM - NA moves into cell
Draws water by osmosis

THEREFORE ECM becomes hyperviscos and high in CL concentration. - Frequently blocked ducts / poor clearance

Basis of chloride sweat test >60mmol

34
Q

What is Kartageners Syndrome?

A

Primary Ciliary Dyskinesia
Presents akin to CF]]failure of clearance of any vesicle lined with cilia

results in bronchiectasis
chronic infection
poor drainage
airway obstruction

35
Q

What is Pickwickian syndrome

A

Form of obesity related lung disease

Cluster of

  1. OSA
  2. Restrictive Pulm function tests
  3. Nocturnal hyerpcapnia
36
Q

How is OSA diagnosed

A
Symptoms:
daytime somnolence
C02 retention headache on waking
poor sleep
witnessed apnoeas
nocturnal waking
Nocturia
Signs
neck collar >17.5cm
shallow breathing pattern >50% - hypopnoea
central cyanosis
facial plethora - secondary polycythaemia
crowded oropharynx
hypo
snoring

Associations:
COPD
Acromegally

Ix:
EPWORTH SLEEPINESS SCALE

37
Q

What is OSA?

A

Obstructive sleep apnoea - apnoeas and hypopnoeas

The increased number of pauses/apnoeas >10secs in normal breathing pattern during sleep and /or reduction in normal tidal volume >50% with desaturation + sleep arousal

50% of OSA in patients who are NOT obese therefore underdiagnosed

38
Q

Investigating OSA

A

OVERNIGHT POLYSOMNOGRAPHY FOLLOWED BY CPAP REPEAT with titration if confirmed.
-sleep lab overnight
A - Continous ECG / EEG / EMG / electro-oculogram
-these determine stage of sleep cycle
B - Resp effort + depth of ventilation

= apnoeas ?central or obstructive

C - microphone - snoring
D - pulse oximetry
E - body position

DIAGNOSIS: Respiratory Disturbance Index = apnoeas/hour

  • > 5 = abnormal
  • 5-15 = Mild
  • 5-30 = Moderate
  • > 30 = Severe
39
Q

Bronchiectasis Community Treatment

A

Education

Physio / Pulm rehab

  • expactoration training
  • PT - chest percussion / / forced expiration / posterior drainage
  • hypertonic saline nebs - CF or Kartageners
  • PEP devices - positive expiratory pressure / oscillating pep to aid removal and clearance of secretions

Vaccination

  • influenza and pneumococcal (FEV1<40% or >65)
  • HiB

Optimise nutrition
-CF / alcohol / a1 antitrypsin / Kartageners may require CREON - start 25,000 with meals and 15,000 snacks

Smoking Cessation

  • 5 As - assess smoker / advise to quit / assess readiness to quit / assist in quiting / arrange follow-up
  • CBT
  • Buproprion
  • Varenicline
40
Q

Bronchiectasis surgical treatment

A

Need very localised disease with otherwise good lung function

  1. LUNG REDUCTION THERAPY
    excision of non functional lung allows for inflation / recruitment of trapped / physiologically normal lung
    REDUCES MORTALITY
    IMPROVES QOL

REQUIREMENTS:
TLCO >20% (diffusion capacity demonstrating functional lung tissue
FEV1 >20%
paC02 <7.3 i.e minimal evidence of T2RF

2. Bullectomy
excision of large bullae
reduces risk of PTx
restores elastic recoil
reduces air resistance and air trapping
increases functional residual capacity

REQUIREMENT:
PROGRESSIVE DYSPNOEA
FEV1<50% - GRADE 3 GOLD
BULLAE >1/3 HEMITHORIAX WITH PRESERVED HISTOLOGY SURROUNDING

  1. Lung Transplant for CF
    1 year survival = 78%
    5 year 51%

REQUIREMENT:
BODE >5 (BMI / MRC dyspn scale 1-5 / GOLD FEV1 (80/50/30) / 6 minute walk test)

41
Q

Bronchiectasis - Medical optimisation

A

Acute phase:

Antibiotics
Frequently colonised pseudomonas
- Tazocin / Ciprofloxacin

Steroids if focally wheezy

Saline nebs
-hypertonic nebs - CF or Kartageners

Aminophyllin may also be indicated

Chronic phase:
Rescue pack of Abx
Prophylactic Abx - regular rotations to avoid MDR
- azithromycin or tobramycin / ciprofloxacin / doxycyclin / amoxicillin

ICS

  • beclometasone
  • fluticasone

Mucolytics

  • carbocisteine
  • glycopyronnium
  • RhDNAse

Antibiotics if needed:
Pseud - fully sensitive = ceftazidime / tobramycin
resistance - pip taz / cipro
Aspergillus - itroconazole and steroid

42
Q

What systems are affected in CF

A
1. Airway
Chronic secretion burden
Poor clearance
Clubbing
Chronic infections
Rapidly colonised
-->Develop bronchiectasis
-permanent dilation of large and middle airways leading to poor drainage / chronic infection and inflammation / airway obstruction
-OBSTRUCTIVE FLOW LOOP
-REDUCED TCO / Kco
  1. Pancreatic insufficiency
    PD atresia / blockade results in Chronic pancreatitis
    -loss of exocrine function and endocrine function
    -Pancreatic autodigestion
    -multiple nutritional deficiencies due to loss of trypsin / amylase / lipase
    -Fatty vitamin deficiency - b12/folate / vit D / Vit E / Vit A
    CREON 25-50,000 TDS + 15-20,000 SNACKS PRN
  • ENDOCRINE INSUFFICIENCY
  • development of DM1
  • insulin
  1. Infertitility
    Juvenile atresia / resorption of Vas Deferens
  2. Meconium ileus
    - fail to pass mec in first 24-48 hours of life
    - bowel obstruction can lead to definitive surgery in first week of life
  3. Focal Biliary Cirrhosis + Portal HTN
  4. Cholestasis and gall stones
  5. Osteoporosis - Vit D and PTH production
  6. Chronic sinusitis / ottitis / nasal polyposis
43
Q

What are common colonisers in CF

A
  1. pseudomonas - late childhood
  2. haemophilus influenza - early pre vacc
  3. staph aureus - breast milk
  4. BURKHOLDERIA CEPACIA COMPLEX
    - RARE
    - ACCELERATES DISEASE BURDEN
    - INCREASE MORTALITY
    - MAY BE A CONTRA-INDICATION TO TRANSPLANT
44
Q

What is BURKHOLDERIA CEPACIA and why is it important?

A

A rare complication of CF is colonisation with BURKHOLDERIA CEPACIA COMPLEX

it accelerates disease burden
may prevent transplant
increases mortality

45
Q

What is the average life expectancy for CF patients

A

AV LE 32 years but increasing to 40 and still active

46
Q

What are the complications of bronchiectasis

A
  1. Recurrent CAP + assoc haemotysis
    - empyema / effusion
  2. Reduced ET and QOL if poorly controlled
  3. Pulmonary HTN - signet ring sign - fibrosed and thickened middle airway adjacent to pulmonary artery
  4. core pulmonale and R sided HF
  5. anaemia of chronic disease
  6. DISSEMINATED INFECTION
    - STAPH SPINAL CORD ABSCESS
    - IE
    - CEREBRAL ABSCESS
  7. SECONDARY AMYLOIDOSIS
47
Q

DEFINE TB

A

ACTIVE / CHRONIC OR PAST INFECTION with mycobacterium Tuberculosis

Acid Fast bacillus
Mitosis in aerobic or anaerobic environments
Very difficult to culture

MDR and VDR forms are increasingly prevalent in Eastern Europe

Typically acute infection presents with severe apical pneumonia with a GOHN focus on CXR which may have an internal fluid level demonstrating encapsulated abscess / caseating internal necrosis

48
Q

What are the complications of old TB

A
  1. Primary reactivation in an immunocompromised state
    - commencing chemotherapy
    - commencing biologic therapy specifically Tcell or anti-TNF
    e. g. infliximab / adalimumab / golimumab- anti-TNFa / vedolizumab - anti a4b7 integrin - T cell homing to gut /
    - HIV or HCV or EBV or HTLV
  2. aspergillosis in old TB cavity
  3. Metastatic infection
    - intestinal TB
    - cerebral TB
    - spinal TB
  4. bronchiectasis
    - Lymphatic compression = poor drainage = bronchial stiffening + infection = fibrosis and stenosis

5.apical lung fibrosis

49
Q

Historic TB treatments - What were they and what signs might you see

A

Theory - TB = air borne therefore aerobic so try and create a hypoxic environment to kill it
FAILED AS TB CAN MITOSE IN AEROBIC + UNAEROBIC

A) Phrenic nerve crush - supraclavicular scar
-aim paralyse diaphragm on affected side to limit ventilation

B) PLOMAGE - thoracotomy scar

  • Similar to pleuredesis but aim to fill chest cavity with inert substance on one side (VS stick pleura together)
  • wax / pingpong balls / gauze

C) THORACOPLASTY - axillary scars / posterior scars / thoracotomy
-Ribs removed to limit expansion

50
Q

TB Signs

A

Chest deformity
Cachexia
high RR / IC or SC recession / Reduced Cricosternal distance
scars - phrenic nerve crush / thoracotomy / drains
Dullness to percusion
Focal crackles
Creps - fine end inspiratory - apical fibrosis or coarse expiratory bronchiectatic
KYPHOSIS - SPINAL TB - POTTS = AKA GIBBUS DEFORMITY

51
Q

Testing For TB

A

AFB cultures x3

TB blood culture

TB genotyping - see if MDR form

Mantoux - Tuberculin skin test
-2 tuberculin units infiltrated SC
-induces delayed hypersensiticity raction - TYPE IV
-T cell mediated
-View results 48-72 hours later
<5mm NEGATIVE
5-14mm Positive
>15mm strong positive
<15mm if prior BCG count as negative

Quantiferon / T-SPOT- IFNy release assay
Uses TB specific proteins and therefore more sensitive and specific than mantoux
-CFP10 / ESAT-6

52
Q

What are some of the proteins used in the IFNy / T SPOT / Quantiferon release Assay for TB

A

CFP10 / ESAT-6 are proteins specific to mycobacterium TB

53
Q

What is Caplan’s syndrome

A

Rheumatoid arthritis + pneumoconiosis

54
Q

What is Loffler’s syndrome

A

Persistent eosinophilia secondary to repeated parasitic infections
results in hypersensitivity
e.g. allergic eosinophillic asthma

classic parasite = ascaris lumbricoides

55
Q

Causes of haemoptysis

A
Vascular
PE
Anti-GBM
rarely IgA nephropathy - case reports
microangiopathic granulomatosis - Wegners

Infective
Pneumonia
TB
invasive aspergillosis - bad sign shows serious invasion

Trauma

iatrogenic
chest drain

metabolic
antiphospholipid syndrome

56
Q

Pulmonary haemorrhage

A

Many causes

Diagnosis:
Haemoptysis
Increased TLCO - reduced expiratory CO as prescence of blood in airway increases diffusion of CO into Hb
High kCO - high transfer coefficient of CO for above reason

look for blood in urine too for Glomerulonephritis
lupus
wegners
anti GBM

57
Q

What are features of mycoplasma pneumonia

A
  1. often severe pneumonia
  2. associated with haemolysis due to AI - anti-I vs rbc
  3. erythema multiforme - target lesions
  4. erythema nodosum - painful IgG deposits
  5. bullous myringitis - blisters on tympani memb - PAIN
58
Q

What is Meliodosis

A

Tropical respiratory infection endemic to Thailand / N Australia causing cavitating pulmonary abscess, pleural effusions, vesicular eruptions over the shins, LFT derangement

MORT 20-50% even with treatment

Organism:
BURKHOLDERIA PSEUDOMALLEI - gram neg bacteria
Burkholderia CAPACCIA ALSO ACCELERATES DISEASE IN CF

treatment:
meropenem + cotrimoxazole

59
Q

What skin changes is squamous cell bronchial carcinoma associated with

A

Erythema gyratum repens

CONCENTRIC ERYTHEMATOUS RINGS LIKE WOOD GRAINS

60
Q

What medications are used for prophylaxis against PCP

A
  1. co-trimoxazole
  2. dapsone

note neither can be used in glucose 6 PD deficiency

  • rash
  • deranged LFTs

Alt:
Nebulised pentamidine

Treatment
Clindamycin

61
Q

Why is Adenosine contra-indicated in Asthma

A
MOA:
Slows conduction through AV node
Targets multiple phsyiological receptors
A1 receptor
-negatively chronotropic
-negatively dromotropic effects 
=Slows AV conduction 
=prolongation of the refractory period

Associtaed with significant bronchoconstriction when inhaled
THEREFORE CONTRA IN ASTHMA
IV VERAPAMIL CAN BE USED

Resus council

  1. vagal maneouvres
    - CS massage
    - bed tilt
    - blow into syringe - forced expiration raises pressure and decreases preload and increases afterload
  2. adenosine 6/12/12
  3. verapamil 2.5-5IV
  4. DC cardioversion
Also contra:
Asthma
sick sinus syndrome
AV and 3rd heart block
ppm
62
Q

How does bronchial carcinoid present?

A

Carcinoid - neuro endocrine tumour
Bronchial carcinoid:
-Arise from Kulchitskys cells in bronchial mucosa
-secrete serotonin

CXR:
Central mass with focal lobular collapse
Tracheal deviation / compression

Presentation:
Flushing / diarrhoea
recurrent chest infections - CP and Wheeze
Hoarse voice - recurrent laryngeal

Pellagra (tumour takes up tryptophan and hypersecretes serotonin) (niacin deficiency)

  • dementia
  • diarrhoea
  • dermatitis

SIADH - confusion / ataxia / hyponatraemia / thirst / fluid overload

Bronchial carcinoid spectrum of NE tumours
-SCLC most malignant
-hypertrophic pulmonary osteoarthropathy
=clubbing and periosteal reaction with wrist pain
-SIADH
-cerebellar degeneration - ANTI-HU antibodies
- CUSHINGS
-Age 50-70

Diagnosis:
HRCT
BAL
Biopsy
Urine 5 hydroxyindoleacetic acid (urinary excreted serotonin metabolite)

Treatment = excision
Symptoms : somatostatin analogues

63
Q

What is Carcinoid Syndrome

A

Carcinoid syndrome describes metastases of carcinoid tumour.

64
Q

Assuming fully sensitive what antibiotic should be used for pseudomonas aeruginosa

A

Ceftazidime 2g IV
Tobramycin

PROPHYLAXIS:
Nebulised Colistin (pyromyxin - mix of colisitin A+B active against gram negative bacilli)
aminoglycosides

RESISTANT:
PIPERACILLIN AND TAZOBACTEM
CIPROFLOXACIN
MEROPENEM AND GENT

65
Q
Describe the patterns of TCO and PFT in
Asthma
COPD
IPF
EAA
pulmonary haemorrhage
PE
A
Remember COPD grading system
GOLD = FEV1/FVC <70%
1 FEV1 >80%
2FEV1 50-80%
3 FEV1 30-50%
4FEV1 <30% or <50% + T2RF
MRC dyspnoea scale
1 no restriction unless strenuous exercise
2 uphill SOB
3 slower pace but makes it
4 stops for breath after 100m
5 house restriced due to SOB

BRODE Index
GOLD + MRC + 6 minute walk test + BMI

Asthma - no reduction in TCO
>15% reversibility on bronchodilator
Normal FVC
Reduced FEV1
FEV1/FVC <70%
Diurnal variation

COPD - reduced TO

  • no or very limited reversibility
  • FVC normal
  • FEV1/FVC <70%
  • expiratory plateau flow loop
  • normal inspiration

IPLF- reduced TO
May show mixed picture fo restriction and obstruction due to pulmonary fibrosis
typically reduced TO and obstructive picture dominates

EAA - reduced TO
Another mixed picture due to allergic obstructive symtoms with superimpose fibrosis - typically apical
does demo some reversibility but beclomet and salbutamol will have a very limited impact
main feature is avoid triggers

Pulmonary Haemorrhage - raised TO
blood in alveoli presents a higher transfer opportunity for CO to displace CO2 and oxygen from haem
anti-GBM / wegners with polyangitis / eosiniophillic granulomatosis and polyangitis (CHURG STRAUSS)
Normal flow loop typically

PE - reduced TO
normal flow loop
VQ mismatch

66
Q

EAA - how may it present

A

Work related variation in airway problems
Wheeze and crackles
intermittent pyrexia

Over time

  • SOBOE
  • restrictive flow loop
  • limited reversibility
  • bronchiectasis and PF
  • clubbing
  • P.HTN + core pulmonale - ilanoprost + sildenafil + p.rehab
  • T2RF
67
Q

What are the sequelae of legionella pneumonia

A
Bibasal crackles
myoglobinuria + proteinuria
hyponatraemia
leucocytosis
LFT derangement

Mx:
macrolides
rifampicin
ciprofloxacin / moxifloxacin

68
Q

What are the sequelae of strep pneumonia

A

Hyponatraemia
gastroenteritis

Post Strep GN
purpura
GN
Arthralgia

Reactive arthritis
GI upset
Arthritis
Urethritis

69
Q

What are the sequelae of mycoplasma pneumonia

A
Severe CXR changes
subacute presentation --> severe T1RF
pleural effusion
pericarditis
GN
Liver synthetic dysfunction
erythema nodosum
erythema marginatum
70
Q

What is postural drainage?

A

Postural drainage is getting in positions that make it easier for mucus to drain. Chest physiotherapy is gently “clapping” parts of the body to remove mucus from the lungs. They are often used together in conditions such as cystic fibrosis or a spinal cord injury (SCI) to help loosen and remove mucus from the lungs.
DAILY FOR BRONCHIECTASIS

71
Q

What is Yellow nail syndrome?

A

Triad of recurrently sterile exudative pleural effusion / lymphadeonpathy and dystrophic yellow nails caused by congenital abnormal lymphatic development

Causes recurrent chest infections due to suppurative ineffective clearance of lymph progressing to bronchiectasis
= subungal oedema / lymphoedema / pleural effusions

72
Q

What is POTTS disease?

One of the oldest diseases to affect mankind

A

TUBERCULOUS SPONDYLITIS
Secondary to extra-spinal infection - usually lungs or mediastinum

Spinal pain
Spinal Rigidity
Spinal Deformity
Cold Abscess
Vertebral collapse and paraplegia

Favours thoracic and lumbar vertebrate

Invasion of Psoas also typically seen

Late Xray changes:

  • Lytic destruction of anterior portion of vertebral body
  • Increased anterior wedging
  • Collapse of vertebral body
  • Reactive sclerosis on a progressive lytic process
  • Enlarged psoas shadow with or without calcification
73
Q

What is Ludwig’s Angina?

A

Life threatening rapidly spreading cellulitis of SUBLINGUAL and SUBMANDIBULAR spaces that starts with an infected lower molar,

If it spreads to SUPRAGLOTTIC tissues–>Risk of airway obstruction

74
Q

What is Young’s syndrome

A

Bronchiectasis
subfertility
Rhinosinusitis

75
Q

How does diaphragmatic weakness present

A

SOBOE
AND
OSA

76
Q

Squamous cell lung cancer is associated with what paraneoplastic process?

A
pancoast tumours are mainly squamous cell
local invasion with late metastases
horners
phrenic nerve involvement
recurrent laryngeal nerve involvement

heavily associated with smoking

PTH related protein
Activates osteoclasts
increases absorption of Ca from PCT and gut
increases phosphate excretion

77
Q

What paraneoplastic syndrome is associated with bronchial adenocarcinoma

A

rarely polymyositis

  • proximal weakness
  • ILD
  • conduction defects
  • foot drop

Anti SRP antibodies

78
Q

SCLC paraneoplastic associations

A

SIADH
CEREBELLAR DEGENERATION AND ATAXIA -anti-Hu Abs
CUSHINGS
-hypertrophic pulmonary osteoarthropathy
=clubbing and periosteal reaction with wrist pain

79
Q

Bronchial carcinoid

A

ACTH ectopic secretion alongside serotonin

note all carcinoid esp gastric associ with pellagra = dementia dermatitis diarrhoea death

80
Q

How is CF treated

A

Prophylactic antibiotics
Creon
postural drainage

LUMACAFTOR
increase translocation of CFTR proteins to the apical surface

IVACAFTOR
increase the number of open CFTR channels

Terlipressin
Modifies pulmonary blood flow and reduces haemotpysis

LAMAs

81
Q

How is IPF treated

A

NON PHARM

Smoking cessation
Pulmonary rehab
- clearance
- exercise
- nutrition
- chest physio
- anxiety management
vaccinations
- influenza
- pneumococcal
oxygen - LTOT
prophylactic antibiotics
lung reduction therapy 
- expand collapsed lung
- bullectomy

PHARM

  • corticosteroids
  • Cyclophosphamide
  • Azathioprine - post transplant
  • Mycophenolate mofetil - post transplant
  • Nintedanib - anti-fibrotic targeting tyr kinases. Shown to slow the decline in lung function in mild-to-moderate IPF.
  • Pirfenidone
82
Q

Describe some biologic agents used in the treatment of asthma

A

Omalizumab - Anti IgE
Where conventional therapy has faied

Mepolizumab - anti-IL-5 - anti - Th2 propogation
used in IgE asthma where conventional therapies have faield

83
Q

What is the difference between montelukast and roflumilast?

A

Montelukast is a leukotriene - 4 antagonist used int he treatment of childhood allerguc asthma as an adjuvant to inhaled therapy

In adults its use is more limited but again as an adjuvant in allergic asthma

Roflumilast is a PDE-4 inhibitor which is used to supplement inhaled therapy in the management of treatment resistant COPD AND BRONCHITIS
- SEVERE WITH FEV1 <50% (GOLD)
- >2 EXACERBATIONS DESPITE BEING ON TRIPLE THERAPY
(LAMA / steroid / LABA)

84
Q

What are the most common forms of Lung Cancer

A

10% of lung cancers are SCLC heavily associated with smoking

  • SIADH
  • CEREBELLAR DEGENERATION - ANTI-HU antibodies
  • CUSHINGS
  • HYPERTROPHIC PULMONARY OSTEOARTHROPATHY

85% are NSCLC comprising

  • adenocarcinoma - no paraneo
  • bronchio-alveolar - no paraneo
  • squamous cell skin cancer - PTHrP - hypercalcaemia

5% Carcinoid
Neuroendocrine
serotonin secreting

Associated with Lambert eaton mysaesthenic syndrome
VGCC Abs
diff from MG as >excitation = >conduction due to >degranulation of ACh granules
Also ?feature of autonomic symptoms like postural drops / incontinence / dry mouth and eyes / erectile dysfunction
Autonomic features are rare in MG

85
Q

What is cold haemolytic anaemia associated with?

A

Mycoplasma pneumonia

see raised LDH
marked reticulocytosis
raised MCV due to high reticulocyte count
low Hb

86
Q

What is the treatment heirachy for acute treatment of asthma exacerbation?

A

B2B salbutamol nebs
ipratropium nebs
IV hydrocortisone
IV magnesium

Limited evidence for aminophyllin in opening airway
Evidence suggesting risk of taccharrythmia outweighs use

non improvement psot these measures would be escalated to ITU for intubation and ventilation

  • PaO2 <8
  • Resp acidosis / rising pCO2
  • Fatigue
  • No improvement or very limited improvement in PEFR

no place for NIV or CPAP

87
Q

What is LEMS?

A

lambert eaton myaesthenic syndrome
50-70% underlying cancer mainly SCLC
https://www.brainscape.com/packs
presents as MG but slightly differently
proximal weakness mainly legs / opthalmoplegia / loss of DTR
1. VGCC abs
2. greater facilitation on repeated contrction VS fatigue
3, greater autonomic features - erectile dys / postual drops / xeropthalmia or xerostoma

88
Q

Pulmonary fibrosis causes

A
Apical
Tb and aspergillosis / aspergilloma
Ank spond
Radiation
Extrinsic allergic alveolitis
Pneumoconiosis
Basal
CTD RA SLE MCTD SYSTEMIC SCLEROSIS
IIP 
Asbestosis
Aspiration
89
Q

Pulmonary fibrosis treatment

A
Steroids
Panther trial proved increasrd comorbidity with azathioprine and steroid
Immunosuppressants
Ciclo RF gum hypertrophy and IS
Bullectomy or lung sparing surgery
FEV1 <50 >20 
TLCO > 20
PAC02 <7.3
Apical disease
Bullae >33% hemidiaphragm
Antifibrotic agents PERFENIDONE for UIP WHEN FEV1 50-80 - poor steroid resp and 2.5 to 5yr mort 
Lung transplant - single lung (scar over good lung and crackles on contralateral side)