Sarcoidosis.
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
Causes of clubbing
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
lung function tests
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
What is diffusion capacity
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
What is total lung capacity and how is it measured
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
explain flow volume loops
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
What is COPD
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
- 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
What is emphysema
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.
What is bronchitis
Bronchitis is defined as a chronic productive cough lasting for 3 months successively over 2 years. It is a form of early COPD
Signs of COPD
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
Grade COPD
- MRC Dyspnoea scale
- FEV1 Gold scale
- 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%
- Mild FEV1>80%
- moderate FEV1 50-80%
- severe FEV1 <50%
- very severe FEV1 <30% OR <50% + T2RF
BODE BMI FEV1 6 Min walk test mrc dyspnoea scale
What is the BODE index
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
How to investigate / diagnose COPD
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
COPD exacerbation causes
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
Smoking Cessation
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
NICOTINE REPLACEMENT THERAPY
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
COPD TREATMENTS
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
COPD Treatment ladder = 3 steps - describe + additional therapies
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
SURGICAL TREATMENT COPD
LUNG REDUCTION
BULLECTOMY
TRANSPLANT
- 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
- Lung Transplant
IMPROVES FUNCTIONAL CAPACITY
1 year survival = 78%
5 year 51%
REQUIREMENT:
BODE >5
CPAP - HOW DOES IT WORK
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
Pneumonia Signs / symptoms
Pneumonia:
X ray evidence of collapse / consolidation within the lung parenchyma
- productive / supporative cough - new
- RR + pyrexia
- tracheal tug . IC recession. SC recession
- T1Rf
- pleuritic CP
- haemoptysis
- Increased diffusion gradient in prescence of pulmonary haemorrhage otherwise reduced
- focal crackles with dullness to percussion with increased vocal phremitis over it ?effusion
- clubbing if chronic / SCLC / bronchiectasis
- effusion / empyema - acidotic septated consolidative effusion
- bronchial breathing
- 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
Pneumonia severity
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
pneumonia investigations
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
pneumonia definition
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
common causes of CAP
Common bacterial
- strep pn up to 40% - esp splenetomy
- haemophilus influenzae - mroe in copd
- mycoplasma - erythema multiforme / marginatum + HAEMOLYSIS + bullous myringitis
- Q fever - chlamydophila
- legionella
- pneumococcal
- staph
- moraxella catarrhalis
- gram negs - aspiration
Atypical
Mycoplasma
pneumococcal - liver dysfunction
chlamydia
C.psitacci
C. burnetti
Viral
influenzae A/B
Treatment pneumonia
- Abx
amox and clarith +/- gram neg cover in aspiration - target sats and appropriate oxygen therapy
- pen Ax - teicoplanin and clarith / flouroquinolone
6 week CXR + FU
consider HIV screen based on presentation
Vaccination
HAP definition
Hospital aquired pneumonia with Sx presenting >72 hours post admission
USually gram neg org / pseudomonas
Define Bronchiectasis
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
Causes bronchiectasis
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
Surgical sieve
V: vascular I: infective/inflammatory T: traumatic A: autoimmune M: metabolic I: iatrogenic/idiopathic N: neoplastic C : congenital
Bronchiectasis imaging changes
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
Specific tests for bronchiectasis
HRCT - definitive
CF GENOTYPING / SWEAT TEST >60mmol CL - AUT REC
KARTAGENERS = primary ciliary dyskinesis - AUT REC
TB - Tuberculin skin test / Quantiferon
Cystic fibrosis - What is the commonest mutation and pathophysiology of the disease process
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
What is Kartageners Syndrome?
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
What is Pickwickian syndrome
Form of obesity related lung disease
Cluster of
- OSA
- Restrictive Pulm function tests
- Nocturnal hyerpcapnia
How is OSA diagnosed
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
What is OSA?
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
Investigating OSA
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
Bronchiectasis Community Treatment
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
Bronchiectasis surgical treatment
Need very localised disease with otherwise good lung function
- 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
- 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)
Bronchiectasis - Medical optimisation
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
What systems are affected in CF
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
- 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
- Infertitility
Juvenile atresia / resorption of Vas Deferens - 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 - Focal Biliary Cirrhosis + Portal HTN
- Cholestasis and gall stones
- Osteoporosis - Vit D and PTH production
- Chronic sinusitis / ottitis / nasal polyposis
What are common colonisers in CF
- pseudomonas - late childhood
- haemophilus influenza - early pre vacc
- staph aureus - breast milk
- BURKHOLDERIA CEPACIA COMPLEX
- RARE
- ACCELERATES DISEASE BURDEN
- INCREASE MORTALITY
- MAY BE A CONTRA-INDICATION TO TRANSPLANT
What is BURKHOLDERIA CEPACIA and why is it important?
A rare complication of CF is colonisation with BURKHOLDERIA CEPACIA COMPLEX
it accelerates disease burden
may prevent transplant
increases mortality
What is the average life expectancy for CF patients
AV LE 32 years but increasing to 40 and still active
What are the complications of bronchiectasis
- Recurrent CAP + assoc haemotysis
- empyema / effusion - Reduced ET and QOL if poorly controlled
- Pulmonary HTN - signet ring sign - fibrosed and thickened middle airway adjacent to pulmonary artery
- core pulmonale and R sided HF
- anaemia of chronic disease
- DISSEMINATED INFECTION
- STAPH SPINAL CORD ABSCESS
- IE
- CEREBRAL ABSCESS - SECONDARY AMYLOIDOSIS
DEFINE TB
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
What are the complications of old TB
- 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 - aspergillosis in old TB cavity
- Metastatic infection
- intestinal TB
- cerebral TB
- spinal TB - bronchiectasis
- Lymphatic compression = poor drainage = bronchial stiffening + infection = fibrosis and stenosis
5.apical lung fibrosis
Historic TB treatments - What were they and what signs might you see
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
TB Signs
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
Testing For TB
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
What are some of the proteins used in the IFNy / T SPOT / Quantiferon release Assay for TB
CFP10 / ESAT-6 are proteins specific to mycobacterium TB
What is Caplan’s syndrome
Rheumatoid arthritis + pneumoconiosis
What is Loffler’s syndrome
Persistent eosinophilia secondary to repeated parasitic infections
results in hypersensitivity
e.g. allergic eosinophillic asthma
classic parasite = ascaris lumbricoides
Causes of haemoptysis
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
Pulmonary haemorrhage
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
What are features of mycoplasma pneumonia
- often severe pneumonia
- associated with haemolysis due to AI - anti-I vs rbc
- erythema multiforme - target lesions
- erythema nodosum - painful IgG deposits
- bullous myringitis - blisters on tympani memb - PAIN
What is Meliodosis
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
What skin changes is squamous cell bronchial carcinoma associated with
Erythema gyratum repens
CONCENTRIC ERYTHEMATOUS RINGS LIKE WOOD GRAINS
What medications are used for prophylaxis against PCP
- co-trimoxazole
- dapsone
note neither can be used in glucose 6 PD deficiency
- rash
- deranged LFTs
Alt:
Nebulised pentamidine
Treatment
Clindamycin
Why is Adenosine contra-indicated in Asthma
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
- vagal maneouvres
- CS massage
- bed tilt
- blow into syringe - forced expiration raises pressure and decreases preload and increases afterload - adenosine 6/12/12
- verapamil 2.5-5IV
- DC cardioversion
Also contra: Asthma sick sinus syndrome AV and 3rd heart block ppm
How does bronchial carcinoid present?
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
What is Carcinoid Syndrome
Carcinoid syndrome describes metastases of carcinoid tumour.
Assuming fully sensitive what antibiotic should be used for pseudomonas aeruginosa
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
Describe the patterns of TCO and PFT in Asthma COPD IPF EAA pulmonary haemorrhage PE
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
EAA - how may it present
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
What are the sequelae of legionella pneumonia
Bibasal crackles myoglobinuria + proteinuria hyponatraemia leucocytosis LFT derangement
Mx:
macrolides
rifampicin
ciprofloxacin / moxifloxacin
What are the sequelae of strep pneumonia
Hyponatraemia
gastroenteritis
Post Strep GN
purpura
GN
Arthralgia
Reactive arthritis
GI upset
Arthritis
Urethritis
What are the sequelae of mycoplasma pneumonia
Severe CXR changes subacute presentation --> severe T1RF pleural effusion pericarditis GN Liver synthetic dysfunction erythema nodosum erythema marginatum
What is postural drainage?
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
What is Yellow nail syndrome?
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
What is POTTS disease?
One of the oldest diseases to affect mankind
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
What is Ludwig’s Angina?
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
What is Young’s syndrome
Bronchiectasis
subfertility
Rhinosinusitis
How does diaphragmatic weakness present
SOBOE
AND
OSA
Squamous cell lung cancer is associated with what paraneoplastic process?
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
What paraneoplastic syndrome is associated with bronchial adenocarcinoma
rarely polymyositis
- proximal weakness
- ILD
- conduction defects
- foot drop
Anti SRP antibodies
SCLC paraneoplastic associations
SIADH
CEREBELLAR DEGENERATION AND ATAXIA -anti-Hu Abs
CUSHINGS
-hypertrophic pulmonary osteoarthropathy
=clubbing and periosteal reaction with wrist pain
Bronchial carcinoid
ACTH ectopic secretion alongside serotonin
note all carcinoid esp gastric associ with pellagra = dementia dermatitis diarrhoea death
How is CF treated
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
How is IPF treated
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
Describe some biologic agents used in the treatment of asthma
Omalizumab - Anti IgE
Where conventional therapy has faied
Mepolizumab - anti-IL-5 - anti - Th2 propogation
used in IgE asthma where conventional therapies have faield
What is the difference between montelukast and roflumilast?
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)
What are the most common forms of Lung Cancer
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
What is cold haemolytic anaemia associated with?
Mycoplasma pneumonia
see raised LDH
marked reticulocytosis
raised MCV due to high reticulocyte count
low Hb
What is the treatment heirachy for acute treatment of asthma exacerbation?
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
What is LEMS?
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
Pulmonary fibrosis causes
Apical Tb and aspergillosis / aspergilloma Ank spond Radiation Extrinsic allergic alveolitis Pneumoconiosis
Basal CTD RA SLE MCTD SYSTEMIC SCLEROSIS IIP Asbestosis Aspiration
Pulmonary fibrosis treatment
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)