apical pulmonary fibrosis

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Respiratory for PACES
Cases for finals
Monday 8th October 2012
Dr James Milburn
Dr Chris Kyriacou
Outline
• Signs to be seen in examination, both expected
and miscellaneous
• Common cases we had/are to be expected in the
exam
– Hx and Ex
– Ix
– Mx
Respiratory Exam
• End of bed inspection
• General Exam
• Chest
–
–
–
–
Inspection
Palpation
Percussion
Auscultation
• Added extras
Inspection (End of bed)
• Observe patient – breathless/comfortable
• Look at surroundings –
inhaler/oxygen/nebulisers etc
• Use of accessory muscles
• Cachexic
General Examination
•
•
•
•
Hands
Face
Neck
Legs
Hands
Hands
Hands
Hands
• Clubbing
– Bronchiectasis, CF, Carcinoma, Fibrosing alveolitis
– 4 signs - FACE
•
•
•
•
Flucance of nail bed
Angle loss
Curvature of nail
Expansion of terminal phalynx
• Tar staining
• Small muscle wasting
– Lung Ca  pressure on brachial plexus
Hands
• HPOA
– Periosteal inflammation in distal ends of long bones
– Primary lung Ca, Meso
• Flap/Tremor
– CO2 retention
– Fine tremor from β2-agonists
• Pulse
– Rate and rhythm
– Bounding
• Cyanosis
Face
Face
Face
• Plethoric
– Secondary polycythaemia, SVC obstruction
• Horner’s (Ptosis, miosis, anhydrosis)
– Pancoast’s, (Demyelination, Carotid aneurysm)
• Anaemia
• Central cyanosis
• Mouth – Halitosis/Thrush
Neck
• Lymphadenopathy
• JVP
Legs
Inspection - Chest
Inspection - Chest
Inspection - Chest
Inspection - Chest
Inspection - Chest
Inspection - Chest
Inspection Chest
Inspection - Chest
• Shape
– Barrel-chested (AP>Lateral)
– Excavatum/Carinatum
• Scars
• Dilated veins
• Ask them to take deep breath
– Reduced expansion
– Symetrical
Palpation
•
•
•
•
Trachea
Apex
Expansion
Vocal fremitus
Percussion
•
•
•
•
Flat – Pleural effusion (thigh)
Dull – Lobar pneumonia (liver)
Resonant
Hyper-resonant –
Emphysema/Pneumothorax
• Tympany – Large pneumothorax (puffed
out cheek)
Auscultation
• Crackles
– Nature of crackles
• Fine – Oedema/Fibrosis (velcro)
• Coarse – Bronchiectasis
– Timing
• Early insp – COPD/Bronchitis
• Mid-late – Fibrosis/Oedema
– Clear on coughing?
• Yes - ?bronchiectasis
• No – Fibrosis/Oedema
Auscultation
• Wheeze
– Inspiratory/Expiratory
– Fixed monophonic - Bronchial Ca
– Polyphonic - Asthma
• Pleural rub
• Vocal resonance
Auscultation
• Breath sounds
– Vesicular – Insp longer than exp
– Bronchial – Exp longer than insp
• Causes of bronchial breath sounds
– Consolidation
– Collapse
– Fibrosis
Back of chest
• Repeat
Added Extras to offer
•
•
•
•
•
Sats
Temp chart
Sputum pot
PEFR
CVS exam
Case 1
• Mrs Jones is 40 yr old women who
presents with a chronic cough
• Please take a history
History
• Cough for last 2 years although now worsening
– No diurnal variation
– No obvious exacerbating factors
• Productive of around ½-1 cupful of foul-smelling
green sputum daily
• Occasional flecks of blood mixed in with sputum
• Had 3 ‘chest infections’ in the last 6 months
• No weight loss
History
• 2 years ago could walk several miles with
no SOB
• During exacerbation is <50yards
• No fever/night sweats
• No chest pain
History
PMH,
• Laparoscopic cholecystectomy 2007
• Whooping cough ~1970
FH,
• Nil of note
Drugs and Allergies,
• Nil
• NKDA
SH,
• Legal secretary for last 15yrs no hx of asbestos exposure
• Ex-smoker for 5 years in her 20’s
• Minimal drinker
• No pets
• No recent travel
Differentials
Differentials
• Bronchiectasis
– Most likely from pertussis as child
– CF unlikely though screen in <40
• Chronic infection
•
•
•
•
COPD – very unlikely without FH of α1-antitrypsin
TB – rule out, no foreign travel, no known exposure
Malignancy – rule out, no wt loss, non-smoker etc
Fibrosis – not dry cough, no occupational risk
Examination
• On examination the patient was clubbed and
had coarse inspiratory crackles bilaterally R>L
• Not dyspnoeic at rest and no use of accessory
muscles.
• A/E and expansion equal
• No wheeze
Investigations
•
•
•
•
Bedside
Bloods
Imaging
Special tests
Bedside
Bedside
•
•
•
•
Sputum
PEFR
Sats
Temperature
Bloods
Bloods
• FBC
– Hb – 10.8
– WCC – 14.2
– MCV – 92
• U+E’s
– Na – 139
– K – 4.1
– Cr – 130
– Ur – 5.2
• CRP – 56.2
Bloods
• FBC
– Anaemia (chronic disease/haemoptysis)
– Polycythaemia (secondary to hypoxia in more advanced
cases)
– Raised WCC if infection
– Eosinophilia if ABPA
• Inflammatory markers – ESR/CRP
• U&E’s
– Renal dysfunction due to amyloid deposition
• Serum immunoglobulins
• Genotyping/Sweat test
Imaging
Imaging
Imaging
Imaging
• CXR
– Flattened diaphragms
– Tramlines from thickened bronchial walls
– Cystic shadows
• CT/HRCT
– Signet rings
– Bronchial wall thickening
Management
Management
• Conservative
• Medical
• Surgical
Conservative
•
•
•
•
Postural drainage
Chest physiotherapy
Pulmonary rehab
Oscillating positive expiratory devices
(Acapella)
Medical
•
•
•
Check for reversibility with β2-agonists
Saline nebs
Vaccinations
•
Little/No role for:
–
–
–
–
Steroids (unless concurrent asthma/COPD)
Human Dnase
Leukotriene agoinsts
Methylxanthines
Medical
• Antibiotics
– Sputum sample before antibiotics
– Choose abx depending on previous
sensitivities
– If previously cultured Pseudomonas need oral
cipro or other IV abx
– Consider low dose macrolides if >3
exacerbations/year
• Macrolides have anti-inflammatory effect
Surgical
• Indicated if localised disease or massive
haemoptysis
• Lobectomy
• Pneumonectomy
Viva-esque Questions
1. Main organisms responsible for infection
in bronchiectasis?
1. H.influezae, S.pneumoniae, Staph
aureus, Pseudomonas, anaerobes
Viva-esque Questions
1. Main organisms responsible for infection
in bronchiectasis?
2. What are the main causes of
bronchiectasis?
1.
H.influezae, S.pneumoniae, Staph aureus, Pseudomonas
2.
Congenital – CF, Kartagener’s, Young’s
Post-infection (childhood) – Measles, pertussis, TB,
Bronchiolitis
Post-infection (adult) – Severe pneumonia, TB
Autoimmune – RA, UC
Obstruction ( localised) – Tumour, Forgien body, lymph node
Idiopathic
Immunocomp – Primary hypogammaglobulinaemia
Traction bronchiectasis – Secondary to fibrosis
Viva-esque Questions
1. Main organisms responsible for infection
in bronchiectasis?
2. What are the main causes of
bronchiectasis?
3. What are the complications of
bronchiectasis?
Viva-esque Questions
3. Infection
Respiratory failure
Brain abscess (haematogenous spread of
infection)
Amyloidosis (renal failure)
Pneumothorax
Viva-esque Questions
1. Main organisms responsible for infection
in bronchiectasis?
2. What are the main causes of
bronchiectasis?
3. What are the complications of
bronchiectasis?
4. What is the definition of bronchiectasis?
Viva-esque Questions
4. Persistent progressive condition
characterised by dilated thick-walled
bronchi. Typically >1.5x the diameter of
the accompanying arteriole
Viva-esque Questions
1. Main organisms responsible for infection in
bronchiectasis?
2. What are the main causes of bronchiectasis?
3. What are the complications of bronchiectasis?
4. What is the definition of bronchiectasis?
5. What are the different morhpological subtypes
of bronchiectasis
Viva-esque questions
5. Cylindrical (uniform calibre and parallel
walls)
Varicose (uncommon – bead like
appearance)
Cystic (severe form where cyst like
bronchi extend to pleural surface)
6. What is Kartagner’s syndrome?
6. Dextrocardia, Bronchiectasis, Chronic
sinusitis
Case 2
• Mr Singh has complained of shortness of
breath
• Please take a history
History
•
•
•
•
•
•
Worsening over last 3 months
Now exercise tolerance <10 yards
Dry cough and pain on coughing
Sleeps with 3 pillows
No haemoptysis
No weight loss
History
PMH,
•
HTN
•
DM
•
Hypercholesterolaemia
Drugs and allergies,
•
NKDA
•
Amlodipine
•
Indapamide
•
Metformin
•
Glicazide
History
FH,
• Nil of note
SH,
• Ex-smoker (20 pack years)
• Around 8 cans strong lager a day
• No travel/pets
• Lives with wife and 2 children
Examination
Examination
• Appears dyspnoeic at rest
• Reduced chest expansion
• B/L lower zone
– Stony dull to percussion
– Absent breath sounds
– Reduced vocal resonance
• No obvious signs of wt loss
• No lymphadenopathy
• No tracheal deviation
Differentials
Differentials
• Pleural effusion
– Secondary to HF
– Secondary to cirrhosis
– Malignancy
• PE
• Fibrosis
Investigations
Bedside
Bedside
• PEFR
• Sats
Bloods
Bloods
•
•
•
•
•
•
•
•
FBC
BNP
U+E
LFTs
CRP
LDH
BNP
Thyroid Function Tests
Imaging
•
•
•
•
CXR
Echo
USS – for guiding drainage
CT (with contrast)/CTPA if ?PE
Imaging
Imaging
• CXR
– Blunting of costophrenic angles
– If larger then opacity with concave upper
margin – Meniscus sign
– Even bigger...complete white out +/mediastinal shift
– Elevated hemidiaphragm if subpulmonic
effusion
What is this....
Pleural fluid analysis
• Transudate <25g/L protein
• Exudate >35g/L
• 25-35g/L
– Exudative if:
• Ratio of pleural fluid to serum protein >0.5
• Ratio of pleural fluid to serum LDH >0.6
• Pleural fluid LDH > 2 thirds of the upper limits of
normal serum value
Pleural fluid analysis
•
•
•
•
Glucose <3.3mmol/L– Malig/Ra/SLE/TB
pH <7.2 – Malig/Ra/SLE/TB
Increased LDH – Malig/Ra/SLE/TB
Increased amylase –
pancreatitis/Carcinoma/Bacterial
pneumonia/Oesophageal rupture
Management
Management
• Conservative
• Medical
• Surgical
Management
• Conservative
Management
• Medical
– BAD ALS (for management of heart failure)
•
•
•
•
•
•
Β-blockers
ACEi
Digoxin
ARBs
Loop diuretics
Spirinolactone
– Pleurodesis – if malignant
Management
• Surgical
– Drainage
• Re-inflation oedema
– Pleurodesis
Rib
Lung
Intercostal
Nerves and Vessels
Intercostal Muscles
Intercostal Space
Fluid (or air)
free in the
pleural cavity
Diaphragm
Viva-esque questions
1. Complications of chest tube drainage
Viva-esque questions
1. Organ damage
Lymphatic drainage  chylothorax
Long thoracic nerve of bell
Rarely arrythmias
Viva-esque questions
2. What are the common causes of a
exudative effusion
Viva-esque questions
2. PRISM
PE
RA
Infection
SLE
Malignancy
Viva-esque questions
3. What are the common causes of
transudative effusions
Viva-esque questions
3.
‘The failures’
Cardiac failure
Nephrotic syndrome
Cirrhosis
Failure to eat – Malabsorption
Viva-esque questions
4. How big does an effusion have to be
before it can be seen on CXR
4. 175-200mls  blunting of C-P angle
Case 3
• Mrs Smith is a 30 year old female who has come
in with a long standing cough
• Please take a history
History
• Cough for last 6 months, remained
relatively constant
• Unproductive of any sputum or blood
• She says she has a constant ‘tightness of
the chest’
• Begun to notice some weight loss
History
• Since the cough began, she has felt more
lethargic with polyarthralgia
• Has recently begun to feel breathless,
even at rest
• Chest pain noted – central, constant,
throbbing, relieved by paracetamol
• Noticed that her eyes feel very itchy and
dry
History
PMH,
• Recurrent conjunctivitis – 2011-12
FH,
• Nil of note
Drugs and Allergies,
• Nil
• NKDA
SH,
• Minimal drinker and non smoker
• No pets, No recent travel
• Work - waitress
Differentials
Differentials
• Sarcoidosis
– Young, female
– Past history of non-pulmonary manifestation of sarcoid
– Cause of apical pulmonary fibrosis
• Malignancy – rule out as weight loss noted, but non smoker,
young
• Extrinsic allergic alveolitis – no occupational exposure
• TB – another cause of pulmonary fibrosis – but no foreign
travel
Examination
• Lupus pernio
– Dusky
– Purple
– Face, Fingers, Feet
• Inspection
– Plaques noted on skin
• Percussion, Palpation – N
• Auscultation
– End inspiratory
– Fine crackles
– APICAL
• Erythema nodosum
– Panniculitis
Viva-esque questions
1. What is sarcoidosis?
Viva-esque questions
• 1. A Multisystem, granulomatous disease
– Of unknown cause
– Scattered collections of granulomas
• Mixed inflammatory cells
• Non-caseating, epithelioid
Viva-esque questions
• 2. What % of patients with sarcoidosis
have pulmonary involvement?
Viva-esque questions
• 2. 90%
– Bilateral hilar lymphadenopathy
– Pulmonary infiltrates
– Fibrosis
Viva-esque questions
• 3. What are the causes of APICAL
pulmonary fibrosis?
Causes of apical pulmonary fibrosis
•
•
•
•
•
•
B – Borelliosis
R – Radiation
E – Extrinsic allergic alveolitis
A – Ankylosing spondylitis
S – Sarcoid
T – Tuberculosis
Case 4
• Mrs Jenkins is a 65 year old female who
has noticed she gets breathless after
walking 50 yards
• Please take a history
History
• Her breathlessness was first noted 6 months ago,
which began after walking 500 yards
• Over the last 2 months this has reduced to 50
yards
• Chronic cough for about 2 years
– Productive of white sputum
• Always has pain in both her hands, but she puts it
down to ‘everyday wear and tear’. Has not sought
medical attention
History
PMH,
• Hypertension
• Hypercholesterolaemia
FH,
• Mother ‘suffered from arthritis’
Drugs and Allergies,
• Amlodipine
• Simvastatin
• NKDA
SH,
• Minimal drinker and non smoker
• Has 2 cats
• No recent travel
• Work – retired lawyer
Differentials
Differentials
• Rheumatoid arthritis
– Older female
– Bilateral long standing small joint arthralgia
– Cause of basal pulmonary fibrosis
• Malignancy – rule out as no weight loss noted, non smoker
• Drug induced – worsening SOB not usually associated with
CCB and Statins
• Scleroderma/CREST – no other extra-pulmonary signs noted
• Asthma – highly unlikely for age, no diurnal variation
Examination
• PIP and MCP affected
• Elbow nodules
• Auscultation
– End inspiratory
– Fine crackles
– BASAL
Viva-esque questions
• 1. What are the pulmonary complications
of rheumatoid arthtitis?
Pulmonary complications of RA
•
•
•
•
•
•
•
Pleural effusion
Nodular lung disease
PULMONARY FIBROSIS
Pulmonary vasculitis
Alveolar haemorrhage
Obstructive pulmonary disease
Infection
Viva-esque questions
• 2. What are the BASAL causes of
pulmonary fibrosis?
Causes of basal Pulmonary Fibrosis
• D – Drugs
– ABC
•
•
•
•
A – Asbestosis
R – Rheumatoid arthritis
S – Scleroderma/Systemic sclerosis
I – Idiopathic pulmonary fibrosis
Viva-esque questions
• 3. What three findings constitute Felty’s
syndrome?
PLUS Neutropenia
PLUS Rheumatoid
arthritis
Investigating Pulmonary fibrosis
Bedside
• Sputum
– ?TB – AFB
• Sats
• Temperature
• Resp rate
Bloods
• FBC
– Hb – 10.0
– MCV - 100
– WCC – 13.2
• Bone profile
– Ca 2.50
• LFT’s
• Rheumatoid factor
• CRP
Imaging
Investigating?
Special tests
•
•
•
•
•
FEV1?
FVC?
FEV1/FVC ratio?
Restrictive or obstructive?
Why?
Lung function
•
•
•
•
•
•
FEV1 Reduced
FVC Reduced
FEV1/FVC ratio same or increased
Restrictive
Why? Decreased lung compliance
Other causes: Obesity, pregnancy, air trapping in
COPD (mixed picture), paralysis/muscle
weakness
Management
Management
• Conservative
• Medical
• Surgical
Conservative
• Oxygen support
• Pulmonary rehab
Medical
• Corticosteroids
– Low dose prednisolone
• Months in duration
• N-Acetylcisteine
• Sildenafil
• Pirfenidone
Surgical
• Lung transplant
– Dependant on
• Severity of pulmonary fibrosis
• Patient health
• Potential improvement
Case 5
• Mr Patel is a 75 year old male with long
term shortness of breath
• Take a history
History
• SOB began 15 years ago, and has been
worsening gradually since
• Now SOB at rest, although previously only
on exertion
• Associated chesty cough
– Productive of ++ sputum
– With associated wheeze
• No weight loss
History
PMH,
• Nil relevant
FH,
• Nil of note
Drugs and Allergies,
• Salbutamol
• Seretide (salmeterol + fluticasone)
• NKDA
SH,
• Started smoking at 25
• Continues to smoke 20 a day
• Drinker in the past, now quit
Differentials
Differentials
• COPD
– Progressive, irreversible airway obstruction
• Cough, SOB, Wheeze
• Long term smoker
• Pneumonia – unlikely, as no acute pathology
• Asthma – unlikely due to age and ++ sputum
Examination
• Inspection
– Barrel chest
– Use of accessory muscles
– Raised RR
• Palpation
– Reduced expansion
• Percussion
– Hyper-resonance
• Auscultation
– Quiet breath sounds
Viva-esque questions
• 1. The term COPD constitutes chronic
bronchitis and emphysema. How would
you recognise each COPD subtype
clinically?
Chronic Bronchitis vs Emphysema
• Obesity
• Frequent, productive
cough
• Accessory muscle use
• Rhonchi
• Wheezing
• Cor pulmonale signs
– Oedema
– Cyanosis
• Thin, barrel chest
• Little/no cough
• PURSED LIP breathing
and accessory muscle
use
• TRIPOD sitting position
• Hyper-resonance
• Wheezing
• Quiet HS
Investigations
Bedside
• Sputum
– Mucoid
– Macrophages typically
• Sats
• Temperature
• Resp rate
Bloods
• FBC
– Raised PCV
• U+E
– Na 147
• a1AT
• BNP?
ABG
•
•
•
•
•
pH 7.40
PO2 8.3
CO2 5.2
BE +1
HCO3 23.4
Investigations?
Lung function
•
•
•
•
•
FEV1?
FVC?
FEV1/FVC ratio?
Restrictive or obstructive?
Why?
Lung function
•
•
•
•
•
•
FEV1 low
FVC normal
FEV1/FVC ratio reduced, LESS than 0.7
Obstructive
Why? Decreased expiratory flow
Other causes? Asthma
Investigations
Management – Chronic COPD
Conservative
• Smoking cessation
– Education
– NRT
– Varenicline
– Bupropion
• Physiotherapy
Medical
• Initial
– SABA (Salbutamol) or SAMA (Ipratropium) prn
• If SOB continues or 2+ exacerbations
– FEV1 >50% (Mild COPD)
• Add LABA (Salmeterol) OR LAMA (Tiotropium)
– If LAMA, STOP SAMA
– FEV1 <50% (Moderate-Severe COPD)
• Add LABA/Steroid combo (Seretide – salmeterol + Flixotide;
Symbicort – formeterol + beclomethasone)
• If exacerbations continue
– Maximise inhaled therapy with LABA/steroid combo + LAMA +
SABA
Medical
•
•
•
•
PO theophylline
PO Carbocisteine
? Oral steroid trial
? Alpha tocopherol ? Beta carotene
Viva-esque questions
• 2. When should long term oxygen therapy
be considered in COPD?
Long term oxygen therapy
• PaO2 <7.3
• PaO2 7.3-8.0 AND
– Secondary polycythaemia
– Nocturnal hypoxaemia – sats <90%
– Peripheral oedema
– Pulmonary hypertension
LTOT
• Supplemental oxygen for at least 15hours
per day
• Greater benefits if 20 hours per day
• Reduces hospital admissions and
frequency of exacerbations
Surgical
• Bullectomy
• LVRS
• Lung transplantation
Acute exacerbations of COPD
Investigations
• Sputum
– Purulent
– Neutrophils
• 3. What organisms commonly can cause an acute
exacerbation of COPD?
•
•
•
•
S. pneumoniae
H. influenzae
M. catarrhalis
P. aeruginosa
Investigations
• Bloods
– FBC
– U+E - ? Effect of theophylline
– CRP
• ABG
–
–
–
–
–
pH 7.30
PO2 7
CO2 7.2
BE -10
HCO3 12
Treatment - Exacerbations
• Oxygen – sats 88-92% - why not higher?
• Antibiotics
– Dependant on organism
• Nebulised bronchodilators
• Oral Prednisolone, to continue as part of
rescue package
• IV aminophylline
• NIV?
Non invasive ventilation
• Persistent hypercapnic ventilatory failure
– T2RF
• No response to medical therapy
• BIPAP can then be used
Case 6
• Mr Baldwin is a 15 year old boy whose
mother is worried about a longstanding
cough
• Please take a history
History
• Cough has lasted around 1 year, worse in
the evenings and in the mornings
• Mr Baldwin has mentioned he feels a
‘band’ around his chest when he needs to
cough, which is dry and hacking
• When this happens, it leaves him very
breathless and wheezy
History
• Also known to have hayfever and eczema,
something that his father also suffers from
Differentials
• Asthma
– Cardinal features - Wheeze, SOB, Cough
– Usually diurnal reversible and variable airflow
obstruction
– Associated atopy and family history
• Aspergillosis – unlikely as no trigger identified,
not diurnal
Examination
• Inspection
– Raised RR
• Palpation
– Hyperinflated chest
• Percussion
– Hyper-resonance
• Auscultation
– Expiratory polyphonic wheeze bilaterally
Investigations
Bedside
• PEFR
• Diary of symptoms/Peak flow
Bloods
• Serum precipitins
Imaging
• Hyperinflation
Special tests
• Spirometry – obstructive picture
– Usually >15% improvement in FEV1 following
SABA or steroid trial
• Skin prick testing
Management of chronic asthma
Viva-esque questions
• 1. What are the aims of asthma treatment,
and what guidelines are they based on?
Viva-esque questions
• 1. British thoracic society guidelines; no
daytime symptoms, no exacerbations, no
rescue medications, lung function >80%
predicted
Conservative
• Removal of any allergens
• Patient education
Medical
• Step 1
– Inhaled SABA prn
• Step 2
– Add inhaled steroid 200-800micrograms/day
• Step 3
– Add inhaled LABA +/- increase inhaled steroid up to
800micrograms/day
• Step 4
– Increase inhaled steroid up to 2000micrograms/day +/leuotriene receptor antagonist, beta agonist PO, MR
Theophylline
• Step 5
– Add long term oral prednisolone
Acute exacerbation of asthma
• Moderate
– PEFR 50-75%
• Severe
– PEFR 33-50%
• Life threatening
– PEFR <33%
Investigating
• Bedside
– PEFR
– Sputum
• Bloods
– FBC, UE, CRP, cultures
– ABG, especially in life threatening
Management of acute asthma
• Oxygen
• Nebulised salbutamol and ipratropium
• Prednisolone 50mg PO
OD/Hydrocortisone 100mg IV QDS
• Call a senior!
• IV Magnesium 1.2-2g infusion
• IV Salbutamol or IV aminophylline
• If numbers not improving  ITU!
Summary
• Signs – common and miscellaneous
• Cases
–
–
–
–
–
Bronchiectasis
Pleural Effusion
Pulmonary fibrosis
COPD
Asthma
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