List I - Core Conditions Flashcards by Patrick Burden (2024)

• Episodic symptoms - more than one of wheeze, breathlessness, chest tightness and cough occurring in episodes
• Wheeze confirmed by a healthcare professional on auscultation
• Evidence of diurnal variability
• Atopic history
• Absence of symptoms, signs or clinical history to suggest alternative diagnosis

• Record as likely to have asthma
• Commence a carefully monitored treatment - typically 6 weeks of ICS
• Assess patient’s status with a validated symptom questionnaire, ideally corroborated with lung function tests (FEV1 or serial peak flows)
• Good response to treatment, confirm diagnosis of asthma
• Poor response to treatment, check inhaler technique and adherence, arrange further tests and consider alternative diagnosis

• Investigate for alternative diagnosis
• Reconsider asthma if the clinical picture changes or an alternative diagnosis is not confirmed
• If reconsidering asthma, undertake or refer for further tests to investigate for a diagnosis of asthma

• Examples include:
• Asthma control questionnaire
• Asthma control test

• Spirometry with bronchodilator reversibility

• Undertake reversibility tests and/or a monitored initiation of treatment assessing the response to treatment by repeating lung function tests and objective measures of asthma control

• Undertake challenge tests and/or measurement of FeNO to identify eosinophilic inflammation

• Watchful waiting with review - for children with mild intermittent wheeze, reasonable to give no maintenance treatment and plan review after an agreed interval period with the parents/carers
• Monitored initiation of treatment for a specified period
• Monitor treatment for six to eight weeks and if there is clear evidence of clinical improvement, continue treatment and diagnose as asthma

• Referral tests not available in primary care
• Red flags and indicators of other diagnoses
• Patient or parental anxiety or need for reassurance

• Prominent systemic features ( myalgia, fever, weight loss)
• Unexpected clinical findings (crackles, clubbing, cyanosis, cardiac disease, monophonic wheeze or stridor)
• Persistent non-variable breathlessness
• Chronic sputum production
• Unexplained restrictive spirometry
• Chest x-ray shadowing
• Marked eosinophilia

• Failure to thrive
• Unexplained clinical findings (focal signs, abnormal voice or cry, dysphagia, inspiratory stridor)
• Symptoms present from birth or perinatal lung problem
• Excessive vomiting or posseting
• Severe upper respiratory tract infection
• Persistent wet or productive cough
• Family history of unusual chest disease
• Nasal polyps

• Age at presentation - early onset wheeze <2yrs the better the prognosis
• Gender - males at greater risk (although boys more likely to grow out of their asthma)
• Coexistence of atopic disease
• Family history of atopy
• Abnormal lung function - persistent reductions in baseline airway function are associated with asthma in adult life

• Current control
• Bronchodilator use
• Validated symptom score
• Time off work/school due to asthma
• Future risk of attacks
• Past history of attacks
• Oral corticosteroid use
• Prescription data
• Exposure to tobacco smoke
• Tests/investigations
• Lung function
• Growth
• Management
• Inhaler technique
• Adherence
• Non-pharmacological management
• Pharmacological management
• Supported self-management
• Education/discussion about self management
• Provision/revision of a written personalised asthma action plan

• Patient education
• Personalised asthma action plan
• Specific advice about recognising loss of asthma control
• Actions to take if asthma deteriorates including emergency help

• Control the disease (defined as):
• No daytime symptoms
• No night-time awakening due to asthma
• No need for rescue medication
• No asthma attacks
• No limitations on activity including exercise
• Normal lung function (FEV1 and/or PEF >80% predicted or best)
• Minimal side effects from medication

1. Start treatment at the level most appropriate to initial severity
2. Achieve early control
3. Maintain control by:
   - Increasing treatment as necessary
   - Decreasing treatment when control is good

• Before starting new drug therapy check adherence with existing therapies, check inhaler technique and eliminate trigger factors

• Clenil Modulite pMDI (Beclometasone dipropionate)
• Very low dose 50mcg x 2 puffs twice a day
• Low dose 100mcg x 2 puffs twice per day
• Medium dose 200mcg x 2 puffs twice per day
• High dose 250mcg x 2 puffs twice per day

• A spacer

• Should be compatible with the pMDI being used
• Drug should be administered by repeated single actuations of the metered dose inhaler into the spacer, each followed by inhalation
• Should be minimal delay between pMDI actuation and inhalation
• Tidal breathing as single breaths
• Spacers should be cleaned monthly rather than weekly or performance is adversely affected - washed with detergent and allowed to dry in the air, the mouth piece should be wiped clean of detergent before use
• Drug delivery via a spacer may vary significantly due to static charge
• Plastic spacers should be replaced at least every 12 months but some may need changing at 6 months

• Consider advising quadrupling ICS at the onset of an asthma attack and for 14 days in order to reduce the risk of needing oral steroids

Anyone with any of the following asthma related features:

• Asthma attack in the last two years
• Using inhaled B2 agonists x 3 per week or more
• Symptomatic x 3 per week or more
• Waking one night a week

• Two to four puffs of salbutamol (100mcg via pMDI and spacer)

• Up to 10 puffs of salbutamol (100mcg via a pMDI and spacer) can be given
• Single puffs should be given one at a time and inhaled separately with five tidal breaths

• 3-4 hours
• If symptoms return within this time further 10 puffs should be given and parents/carers should seek urgent medical advice

• Tobacco smoking - Cigarette smoking most common risk factor (90%)- Risk is also increased with pipe, cigar, water pipe and maijuana smoking
• Occupational exposure (20%)- Dust - coal, grains, silica- Chemicals - welding fume, isocyanates and polycyclic aromatic hydrocarbons
• Air pollution- Indoor air pollutants from burning wood and biomass- Role of outdoor pollutants is unclear
• Genetics- Alpha 1 anti-trypsin deficiency, typcially presents in younger age patients (<45 years) , affects smokers and non-smokers
• Lung development- Factors affecting lung growth and development (maternal smoking and pre-term birth) and in childhood (severe respiratory tract infection and passive smoking) have been associated with reduced lung function and potentially increased risk of COPD in adulthood
• Asthma- One study found a 12 fold higher risk of patients with asthma developing COPD compared to those without

• Common, treatable (not curable) and largely preventable lung condition
• Characterised by persistent symptoms (breathlessness, cough, and sputum) and airflow obstruction (usually progressive and not fully reversible)
• Obstruction results from chronic inflammation caused by exposure to noxious particles or gases (usually tobacco smoke but also from environmental and occupational exposures)
• COPD is a triad of chronic bronchitis, emphysema and COPD
• Emphysema - loss of parenchymal lung texture
• Chronic bronchitis - clinical term for cough and sputum production for at least 3 months in each of 2 consecutive years
• Exacerbations are acute episodes of worsening COPD symptoms (such as increased SOB, cough and sputum beyond normal day to day variations)

• Chances of developing COPD can be significantly reduced if you avoid smoking
• If you already smoke, stopping can prevent further damage
• For help to stop smoking contact - NHS Smokefree or ask the GP about stop smoking treatments available

• Based on typical clinical features and supported by spirometry

• Suspect COPD in people over the age of 35 with a risk factor such as smoking, occupational or environmental exposure and one or more of the following:
• SOB - persistent, progressive over time and worse on exertion
• Chronic/recurrent cough
• Regular sputum production
• Frequent lower respiratory infections
• Wheeze

Other symptoms may be present:

• Weight loss, anorexia, fatigue
• Waking at night with SOB
• Ankle swelling - consider cor pulmonale
• Chest pain
• Haemoptysis- Reduced exercise tolerance

• Cyanosis
• Raised JVP
• Cachexia
• Hyperinflation of the chest
• Use of accessory muscles and/or pursed lip breathing
• Wheeze and/or crackles of the chest

• FEV1/FVC <0.7 confirms persistent airflow obstruction
• Consider other causes in older people without typical symptoms of COPD who have FEV1/FVC <0.7
• Consider COPD in younger people who have symptoms of COPD even when FEV1/FVC ratio is >0.7
• Consider alpha1-anti-trypsin deficiency if the person is younger than 40 years of age or has a family history
• Be aware that COPD can exist with other conditions

• Onset, variability and progression of:
• Breathlessness
• Cough and sputum production
• Peripheral oedema - consider cor pulmonale
• Weight loss
• Exposure to risk factors including:
• Smoking
• Occupational exposures
• Impact of symptoms on daily life and occupation - COPD assessment test
• Previous exacerbations or hospitalisation
• Past medical history and comorbidities including
• Anxiety and depression
• Cardiovascular disease and metabolic syndrome
• Lung and liver disease
• Osteoporosis- Asthma
• Family history - Lung or liver disease
• Consider underlying alpha
• 1 anti-trypsin deficiency

• General examination - vital signs - HR, RR, temp, BP, O2 sats
• Examine the chest and check for peripheral oedema and other signs of cor pulmonale
• Measure weight and height to calculate BMI (kg/m2)

• Chest x-ray - to help exclude other causes (lung cancer, bronchiectasis, tuberculosis and heart failure)
• Full blood count - to identify anaemia or polycythemia
• Spirometry - measure post bronchodilator spirometry to confirm the diagnosis of COPD - do not perform reversibility testing as part of the work up
• BMI

• Sputum culture - if purulent and persistent
• Serial home peak flow
• ECG and serum natriuretic peptides - if cardiac disease or pulmonary hypertension are suspected - echocardiogram may also be indicated
• CT thorax - if symptoms seem disproportionate to spirometry measurements, another diagnosis (fibrosis or bronchiectasis) is suspected, or an abnormality on chest x-ray requires further investigation
• Serum alph1-anti-trypsin- Consider in people with early onset symptoms, minimal smoking history or a positive family history
• Referral to a specialist for management and screening of family members is required if alpha-1 antitrypsin deficiency is identified

• Post bronchodilator spirometry should be performed and interpreted by an appropriately trained heath care professional to confirm the diagnosis of COPD
• Spirometry should be carried out 15-20 minutes after the person has inhaled a short acting bronchodilator (e.g. 400mcg salbutamol via a spacer)
• Airflow obstruction is defined as post bronchodilator ratio of FEV1/FVC <0.7
• Routine spirometry reversibility testing is not recommended
• Spirometry should be performed at diagnosis, when diagnosis is reconsidered and for monitoring of disease severity and progression

• Stage 1 - mild - FEV1 80% of predicted value or higher
• Stage 2 - moderate - FEV1 50-79% of predicted value
• Stage 3 - severe - FEV1 30-49% of predicted value
• Stage 4 - very severe - FEV1 <30% predicted value or FEV1 <50% with respiratory failure

• Asthma
• Bronchiectasis
• Heart failure
• Lung cancer
• Interstitial lung disease
• Anaemia
• Tuberculosis
• Cystic fibrosis
• Upper airway obstruction

• Test to measure the impact of COPD on a persons daily life
• Scored 1-5 per question
• Cough
• Phelgm/mucus
• Chest tightness
• Activity and SOB
• Activity limitations
• Confidence leaving home
• Sleep
• Energy levels
• Measure of disease progression - not for diagnosis

• MRC dyspnoea scale
  1. Not troubled by breathlessness except during strenuous exercise
  2. Short of breath when hurrying or walking up a slight hill
  3. Walks slower than contemporaries on the level because of breathlessness, or has to stop for breath when walking at own pace
  4. Stops for breath after walking about 100 m or after a few minutes on the level
  5. Too breathless to leave the house, or breathless when dressing or undressing

• Sustained worsening of symptoms from their usual stable state (beyond normal day to day variations) which is acute in onset
• Can be triggered by a range of factors - respiratory tract infections (commonly rhinovirus), smoking, and environmental pollutants
• Common symptoms include:
• Increased breathlessness
• Increased cough
• Increased sputum production and change in sputum colour
• Other symptoms include
• Increased wheeze and chest tightness
• Upper respiratory tract symptoms
• Reduced exercise tolerance
• Ankle swelling
• Increased fatigue
• Acute confusion

• Pneumonia
• Pulmonary embolism
• Pneumothorax
• Acute heart failure
• Pleural effusion
• Cardiac ischaemia or arrhythmia
• Lung cancer
• Upper airway obstruction

• Explain the diagnosis, risk factors for progression and the importance of healthy diet and physical activity, patient information is available from:
• British lung foundation
• NHS
• COPD
• Offer treatment and support to stop smoking at every opportunity
• Offer pneumococcal and influenza vaccinations
• Offer pulmonary rehabilitation if indicated
• Develop a personalised self management plan with the person
• Optimise treatment for comorbidities
• Screen for anxiety and depression

• Offer a short acting beta 2 agonist (SABA) or short acting muscarinic antagonist (SAMA - ipotropium) to use as needed to relieve breathlessness and improve exercise tolerance
• Ensure the person has appropriate training on use and can demonstrate satisfactory technique
• Regularly review medication, adherence and inhaler technique

• Review to ensure that non-pharmacological management is optimal and they are up to date with vaccinations
• Make sure symptoms are not due to another cause
• If no asthmatic features or features suggestive of steroid responsiveness:
• Offer a long acting beta 2 agonist (LABA - salmetrol) plus a long acting muscarinic antagonist (LAMA - tiotrophium)
• If the person continues to have symptoms day to day adversely affecting their life:
• Consider 3 month trial of LABA + LAMA + ICS - No improvement in 3 months change back to LABA + LAMA
• If symptoms have improved, continue with LAMA + LABA + ICS and review at least annually
• If the person has asthmatic features suggestive of steroid responsiveness consider offering LABA + ICS
• If the person continues to have day to day symptoms adversely affecting quality of life or has 1 severe (need hospitalisation) or 2 moderate exacerbations of COPD within a year, offer LABA + LAMA + ICS

• Increased risks (including pneumonia)

* Clearly document the reasons if they are treated with ICS

• Lung cancer suspected - haemoptysis or suspicious features on chest x-ray
• Diagnostic uncertainty - difficulty distinguishing COPD from asthma
• COPD is very severe or rapidly worsening - e.g. FEV1 <30% * Cor pulmonale is suspected
• <40 years or family history of alpha1 - antitrypsin deficiency
• Frequent infections - to assess preventable factors and exclude bronchiectasis
• Assess the need for:
• Oxygen therapy
• Long term non-invasive ventilation
• Nebuliser therapy or long term oral corticosteroids
• Lung surgery - person with bullous lung disease who is still symptomatic on maximal treatment

• If they are functionally disabled by COPD - MRC grade 3 of above or have had recent hospitalisation for an acute exacerbation
• Advise that commitment to pulmonary rehabilitation can improve quality of life, increase exercise capacity and reduce breathlessness
• Do not refer to pulmonary rehabilitation if
• Unable to walk
• Unstable angina, or have had a recent myocardial infarction

• Do not start oxygen therapy without a specialist assessment
• Oxygen is a treatment for hypoxaemia (not breathlessness)
• Long term oxygen therapy can improve survival in people with stable COPD and chronic hypoxia
• Inappropriate O2 therapy in people with COPD may cause respiratory depression

• Refer the person for LTOT assessment if they have:
• O2 sats of 92% of less breathing air
• PO2 <7.3
• Very severe (FEV1 <30% predicted or severe FEV1 30-49% predicted airflow obstruction
• Cyanosis
• Polycythaemia
• Peripheral oedema
• Raised JVP

Refer for assessment for abulatory oxygen therapy (portable) people on LTOT who are mobile outdoors

• Do not offer short burst oxygen therapy for breathlessness in people with COPD who have mild or no hypoxaemia at rest
• Palliative oxygen therapy may be considered by a specialist for people with intractable breathlessness which is non-responsive to other treatment
• Warn people on oxygen not to smoke because the risk of fire or explosion

• Consider referral to physiotherapist for a person with excessive sputum to learn:
• How to use positive expiratory pressure devices
• Active cycle of breathing techniques
• Consider referral to social services and occupational health if:
• Person is experiencing difficulties with activities of daily living
• Consider referral for dietetic advice if:
• BMI is abnormal (high or low) or changing over time (3 kg or more in an older person)
• Consider referral to psychological services if:
• Anxiety or depression related to COPD are identified

• Via an inhaler

• Via a spacer

• Oral corticosteroids
• Oral theophylline (slow release)
• Oral mucolytic therapy
• Oral anti-tussive therapy
• Oral prophylactic antibiotic therapy
• Oral phosphodiesterase-4 inhibitors

• Should be developed in collaboration with the person
• Provide personalised information and advice on:
• COPD and symptoms
• Non-pharmacological measures including diet, physical activity, pulmonary rehabilitation, smoking cessation and avoidance of passive smoking
• Importance of vaccinations
• Appropriate use of inhaled therapies (including inhaler technique and adherence)
• Early recognition and management of exacerbations
• How to adjust SABA therapy
• When to take short courses of steroids and antibiotics to keep at home for exacerbations
• When to contact a health care professional
• Details of local and national organisations and online resources
• Review self management plans regularly

Assess for features of the following:

• Worsening breathlessness
• Increased sputum volume and purulence
• Cough
• Wheeze
• Fever without obvious source
• Upper respiratory tract infection in the past 5 days
• Increased respiratory rate or heart rate increase 20% above baseline

Severe exacerbation may be suggested by:

• Marked breathlessness and tachpnoea
• Pursed lip breathing and/or use of accessory muscles at rest
• New onset cyanosis or peripheral oedema
• Acute confusion or drowsiness
• Marked reduction in ADL’s Clinical assessment
• Vital signs
• Assess for confusion AMTS
• Examine the chest
• Check ability to cope at home
• Consider the need for hospital admission
• Do not send sputum samples for culture routinely
• Consider other causes of symptoms (MI, worsening heart failure, pulmonary embolus and pneumonia)

Consider emergency admission if the person has any of the following:

• Severe breathlessness
• Inability to cope at home (or living alone)
• Poor or deteriorating general condition including significant comorbidity (cardiac disease, diabetes)
• Rapid onset symptoms
• Acute confusion or impaired consciousness
• Cyanosis
• Oxygen sats <90%
• Give O2 if awaiting emergency transfer
• Otherwise give patients with COPD oxygen via a venturi 24% mask at 2-3 l/min or venturi 28% at a flow rate of 4l/min or nasal cannula at a flow rate of 1-2 l/min
• Target sats at 88-92% in most cases
• Worsening peripheral oedema
• New arrhythmia
• Failure of exacerbation to respond to initial treatment
• Already receiving long term oxygen therapy
• Changes on x-ray

• Advise the person to increase the dose or frequency of their SABA (without exceeding the maximum dose)
• Consider oral corticosteroids for people with a significant increase in breathlessness that interferes with daily activities
• 30 mg oral prednisolone once a day for 5 days
• Consider need for osteoporosis prophylaxis for people requiring 3-4 courses of corticosteroids per year)
• Consider the need for antibiotic treatment taking into account:
• Severity of symptoms (sputum colour changes, volume, thickness)
• Risk of complications - Previous sputum culture and susceptibility results
• Risk of antimicrobial resistance and current prophylaxis

• First choice antibiotics include:
• Amoxicillin 500 mg x 3 per day for 5 days
• Doxycline 200 mg on first day, then 100 mg once a day for 5 day course in total
• Clarithromycin 500 mg x 2 per day for 5 days
• If no improvement on first choice taken for 2 to 3 days
• Send sputum sample for culture and susceptibility testing
• Offer an alternative first choice antibiotic from a different class
• If the person is a high risk of treatment failure - e.g. resistant bacteria consider prescribing co-amoxiclav 500/125 mg x 3 per day for 5 days

• Reassess people with an acute COPD exacerbation if their symptoms worsen rapidly or significantly
• Consider other diagnoses
• Symptoms or signs of a more serious illness
• Antibiotic resistance
• Need for admission
• Send sputum sample for culture and sensitivity testing if symptoms have not improved following antibiotic treatment and it has not already been done
• Follow up all people who have had an acute exacerbation of COPD when they are clinically stable (e.g. 6 weeks after onset of exacerbation)
• Assess residual or changed symptoms and need for further investigations (chest x-ray)
• Optimise non-pharmacological and pharmacological management
• Ensure the person knows how to use the prescribed medications
• Consider the need for referral to respiratory specialist
• Offer a short course of oral corticosteroids and short course of antibiotics to keep at home as part of the persons exacerbation plan if:
• Have had an exacerbation in the last year and remain at risk
• Understand and are confident how to take the medications and are aware of the associated risks and benefits
• Know when to seek help and when to ask for replacements once medication has been used
• 3 or more courses of corticosteroids and/or oral antibiotics in the last year investigate the possible reasons for this
• Review the persons self management plan

• No commonly accepted definition
• For most there will be a gradual decline punctuated by acute exacerbations which increase the risk of dying
• Factors associated with increased risk of mortality from COPD include:
• Frequency and severity of exacerbations
• Hospitalisation during an exacerbation
• Poor lung function on spirometry
• Low BMI
• Comorbidities such as CV disease and malignancy
• Gold standards framework may be useful to identify those who are approaching the end of their life

• Focus is on palliative care to relieve symptoms and improve quality of life
• Ensure the person has an advance care plan (if they wish) and discuss end of life issues (where appropriate) including advance decisions
• Coordinate with a respiratory nurse specialist, district nurse, palliative care team, and social services as appropriate
• Optimise treatment associated with COPD such as:
• Breathlessness - keeping the room cool, improving air circulation, opiates, oxygen
• Cough
• Secretions
• Pain
• Insomnia
• Depression
• Anxiety

See palliative care management

• Understanding of their illness and prognosis
• Concerns and preferences for future treatment and care
• Preferred place of care
• When, who and how to call for help when there is a crisis or acute exacerbation and management options
• Discontinuation of inappropriate interventions
• Interventions which might be considered in an emergency - anticipatory medicines
• CPR discussion if they were in a life threatening deterioration
• Support of family and carers
• Needs for psychological and spiritual care
• Written plan in their home available with them if admitted to hospital, care home or hospice
• Advance decisions if appropriate

• Continued smoking or relapse for ex-smokers
• Exposure to passive smoke
• Viral or bacterial infection
• Indoor and outdoor air pollution
• Lack of physical activity
• Seasonal variation (winter and spring)

• Lung function tests FEV1
• ASA grade
• Optimise medical management of people with COPD before surgery if time permits - e.g. pulmonary rehabilitation

• At least annually

• Smoking status and motivation to quit
• Adequacy of symptom control:
• Breathlessness
• Exercise tolerance
• Estimated exacerbation frequency
• Need for pulmonary rehabilitation
• Presence of complications
• Effects of each drug treatment
• Inhaler technique
• Need for referral to specialist and therapy services
  Measurements
• FEV1 and FVC
• Calculate BMI
• MRC dyspnoea score

• At least twice a year

• Smoking status and motivation to quit
• Adequacy of symptom control:
• Breathlessness
• Exercise tolerance
• Estimated exacerbation frequency
• Presence of cor pulmonale
• Need for long term oxygen therapy
• Nutritional state
• Presence of depression
• Effects of each drug treatment
• Inhaler technique
• Need for social services and occupational therapy input
• Need for referral to specialist and therapy services
• Need for pulmonary rehabilitation

Measurements

• FEV1 and FVC
• Calculate BMI
• MRC dyspnoea score
• O2 sats

• Measure spirometry in all people before discharge
• Re-establish people on their optimal maintenance bronchodilator therapy before discharge
• People with an episode of respiratory failure should have satisfactory oximetry or arterial blood gas results before discharge
• Assess all aspects of routine care that people receive including appropriateness and risk of side effects before discharge
• Give people information to enable them to understand the correct use of medications, including oxygen before discharge
• Make arrangements for follow up and home care (visiting nurse , oxygen delivery or referral for other support before discharge
• Make sure that the person, their family and the physician should be confident that they can manage successfully before they are discharged - formal activities of daily living assessment may be helpful when there is still doubt

• Previous or existing diagnosis of asthma or of atopy
• Higher blood eosinophil count
• Substantial variation in FEV1 over time (at least 400 ml) or substantial diurnal variation in peak expiratory flow (at least 20%)

• Right sided heart failure secondary to lung disease and is caused by pulmonary hypertension as a consequence of hypoxia

• Peripheral oedema
• Raised JVP
• Systolic parasternal heave
• Loud pulmonary second heart sound (over the second left intercostal space)
• Hepatomegaly

Other causes of peripheral oedema should be considered

• Individually tailored, multidisciplinary care program for people with COPD which aims to optimise physical and psychological condition through training, education and nutritional, psychological and behavioural interventions
• 6-12 week programme, 2 times weekly

• Group 1 (car or motorcycle) or group 2 (lorry or bus) - DVLA need not be informed unless any complications are associated with cough, syncope, disabling dizziness, fainting or loss of consciousness
• If the DVLA needs notifying advise the person that it is their responsibility to do so

• Haematocrit - raised
• RBC - raised

Impaired O2 exchange in the lungs can result in a low PaO2 which can stimulate EPO release from the kidneys - EPO stimulates erythrompoiesis and increases red cell mass thereby resulting in polycythaemia

• Using FEV1 (of predicted)
• > 80% - Stage 1 mild
• 50-79% - Stage 2 moderate
• 30-49% - Stage 3 severe
• <30% - Stage 4 very severe

Plus post bronchodilator FEV1/FVC <0.7

• Alpha-1 antitrypsin (A1AT) deficiency

• Haemophilus influenzae (most common cause)
• Streptococcus pneumoniae
• Moraxella catarrhalis

• If the patient has had maximal standard medical therapy in the form of nebulisers, steroids and theophylline, non-invasive ventilation (NIV) should be considered

• COPD with respiratory acidosis pH 7.25-7.35 (can also be used in patients who are more acidotic (i.e. pH <7.25) but that a greater degree of monitoring is required (e.g. HDU)
• Type II respiratory failure secondary to chest wall deformity, neuromuscular disease or obstructive sleep apnoea
• Cardiogenic pulmonary oedema unresponsive to CPAP
• Weaning from tracheal intubation

• Expiratory Positive Airway Pressure (EPAP) 4-5 cm H2O * Inspiratory Positive Airway Pressure (IPAP) RCP advocate 10 cm H2O - BTS suggest 12-15 cm H2O
• Back up rate 15 breaths per minute
• Back up inspiration:expiration ratio: 1:3

• At least 15 hours per day

Assess patients if any of the following:

• Very severe airflow obstruction (FEV1 <30% predicted)
• Considered for patients with severe airflow obstruction (FEV1 30-49% predicted)
• Cyanosis
• Polycythaemia
• Peripheral oedema
• Raised JVP
• Oxygen sats <92% on room air

• Measuring arterial blood gases on 2 occasions at least 3 weeks apart in patients with stable COPD on optimal management
• Offer LTOT to patients with a pO2 of <7.3 kPa or to those with a pO2 of 7.3 - 8 kPa and one of the following:
• Secondary polycythaemia
• Peripheral oedema
• Pulmonary hypertension
• Do not offer LTOT to people who continue to smoke despite being offered smoking cessation advice and treatment, and referral to specialist stop smoking services
• NICE suggest a structured risk assessment
• Risk of falls tripping over the equipment
• Risk of burns and fires , and the increased risk of these for people who live in homes where someone smokes (including e-cigarettes)

• Azithromycin is recommended for selected patients
• Patients should not have smoked, should have optimised standard treatments and continue to have exacerbations
• Patients require a CT thorax (to exclude bronchiectasis) and sputum culture (to exclude atypical infections and tuberculosis)
• LTFs and an ECG to exclude QT prolongation should also be done as azithromycin can prolong QT interval

• Type 2 - e.g. COPD

• Type 1 - e.g. pulmonary oedema

• Air entry into the pleural space without causing mediastinal shift and tracheal deviation

• Spontaneous - no evidence of precipitating event 1st degree if no underlying lung disease, 2nd degree if there is evidence
• Acquired - precipitating cause identified

Spontaneous

• 1st apircal/subpleural bulla
• 2nd COPD, pneumonia, lung Ca, asthma, pulmonary fibrosis, CF, TB, lung abscess, sarcoidosis, connective tissue disorders Marfans, Ehlers-Danlos

Acquired

• Traumatic, iatrogenic - central line insertion, pleural aspiration/biopsy, percutaneous liver biopsy, barotrauma (ventilation)

• Male
• Young
• Thin

• 17/100,000/yr
• Biphasic 15-34yrs 1st, >55yrs 2nd)
• M>F 2.5:1
• No seasonal variation

• 1st/2nd degree - asymptomatic (fit, young, small pneumothorax) or sudden onset ipsilateral pleuritic chest pain, SOB (severity depends on size)
• 2nd degree - sudden deterioration in underlying lung disease
• Acquired - recent trauma, reduced o2 sats, increased ventilation pressures, barotrauma
• HPC - at rest (most spontaneous resolve <24 hrs, +/- resolution of the pneumothorax, after injury

• SOB, increased HR
• Reduced chest expansion
• Hyper-resonant percussion
• Reduced breath sounds and reduce VR
• Look for signs of underlying lung disease

• Tension pneumothorax

* Large emphysematous bulla on CXR

• ABG (if SOB or patients with chronic lung disease)

* Expiratory chest x-ray - thin pleural edge as faint line, loss of lung markings distal to it

• Lung re-expansion

* Prevention of recurrence

• If the rim of air is <2cm and the patient is not short of breath then discharge should be considered
• Otherwise aspiration should be attempted
• If this fails (defined as >2cm or still SOB) then a chest drain should be inserted
• Patients should be advised to stop smoking to reduce the risk of further episodes - lifetime risk of developing a pneumothorax in healthy smoking men is around 10% compared with around 0.1% in non-smoking men

• If the patient is >50 years old and the rim of air is >2cm and/or the patient is SOB then a chest drain should be inserted
• Otherwise aspiration should be attempted if the rim of air is between 1-2cm
• If aspiration fails (i.e. pneumothorax is still greater than 1cm) a chest drain should be inserted
• All patients should be admitted for at least 24 hours

Pre-procedure

• Infiltrate 2nd ICS MCL with 10ml 1% lidocaine to pleura overlying pneumothorax

Procedure

• Insert a 16G cannula into the pleural space, remove needle, connect 3 way tap and 50ml syringe, aspirate up to 2.5L of air, stop if resistance is felt/patient is coughing excessively

Post procedure

• CXR at 24h and 7-10 days to exclude recurrence

Pre-procedure

• Identify site (CXR/CT/USS);

Procedure
* Infiltrate 10-20mL 1% lidocaine (in “safe triangle”), ensuring air/fluid aspirated → 2cm incision above rib (avoids NV bundle below) → blunt dissection to pleura → puncture with forceps → insert chest drain (± Seldinger technique) → advance upwards towards apex → attach drain to underwater seal (ensure it is bubbling with respiration) → close incision of large with mattress suture (purse-string no longer recommended as risk scarring/wound pain);

Post-procedure
* NEVER clamp a bubbling chest drain → CXR straight after (to confirm position) → remove (during expiration/Valsalva) 24h after lung re-expansion on CXR and stopped bubbling

• Thoracic/abdominal wall injury
• Chylothorax
• Winged scapula (damaged long thoracic nerve)
• Arrhythmia (rare)
• Persistent bubbling air leak from the lung
• Blocked tube from the clots/kinking

• Bilateral pneumothoraces
• Lung fails to re-expand with intercostal drain >2 previous pneumothoraces on the same side, previous pneumothorax on the opposite side

• Risk of progression to tension pneumothorax (uncommon)
• Resolution rate of 1% in 24h
• Aspiration success rate of 55% but more patients with air leak at 10 days vs chest drain

• Avoid air travel for 6 weeks after a normal chest x-ray

* Avoid driving forever

• Fluid in the pleural space
• Haemothorax - blood
• Empyema - pus
• Chylothorax - chyle: lymph with fat
• Haemopneumothorax - blood and air

• Transudates
• Protein <30g/l
• Exudates
• Protein >30g/l

This distinction is not accurate when serum protein is abnormal or when the pleural fluid protein is close to 30g/l - in this situation Light’s criteria is used

• Light’s criteria may be applied when protein is between 25-35 g/l
• Pleural fluid is considered exudate if one of the following criteria are met:
• Pleural fluid protein divided by serum protein is >0.5
• Pleural fluid lactate dehydrogenase (LDH) divided by serum LDH is >0.6
• Pleural fluid LDH >2/3 the upper limits of laboratory normal value for serum LDH

• Heart failure (most common transudate cause)
• SVCO
• Hypoalbuminaemia (liver disease, nephrotic syndrome, malabsorption)
• Hypothyroidism
• Meig’s syndrome - R pleural effusion, ascites and ovarian fibroma

• Infection - pneumonia (most common exudate cause), TB subphrenic abscess
• Connective tissue disease - RA, SLE
• Neoplasia - lung cancer, mesothelioma, metastases
• Pancreatitis
• Pulmonary embolism
• Dressler’s syndrome
• Yellow nail syndrome

Transudate
* HF, liver cirrosis, hypoalbuminaemia

Exudate
* Pneumonia, malignancy

• Increase in hydrostatic pressure vs oncotic pressure

• Increased capillary permeability from infection/inflammation/malignancy

• Asymptomatic
• SOB
• Pleuritic chest pain

• Bilateral - transudate
• Unilateral - exudate
• Reduced chest expansion, aspiration marks, tracheal deviation away from affected side - large effusion, reduced TVF
• Stony, dull percusion
• Reduced air entry, diminished breathsounds, bronchial breathing above the effusion (where the lung is compressed), reduced VR
• In malignancy - cachexia, clubbing, lyphadenopathy, radiation marks, mastectomy scars
• Chronic liver disease, HF, hypothyroidism, RA, SLE (butterfly rash)

• Pneumothorax - reduced chest expansion, tracheal deviation
• COPD - hyper-inflated barrel chest
• Pneumonia - reduced chest expansion, dull percusion
• Lobar collapse - reduce chest expansion, normal percussion, reduced air entry

• LFT - albumin - to compare with pleural, LDH (to compare with LDH)
• CXR - (PA and lateral)
• Small effusions - at least 200ml - blunt costophrenic angles
• Large effusions - at least 500ml - obscure hemi-diaphragms, water dense shadows, concave upper poles
• CT chest if initially fail to identify the cause
• USS guided pleural tap - if clinically significant effusion of unknown cause (not if bilateral)

Procedure

• Percuss the upper border of effusion (usually posterior/lateral) and infiltrate 5-10ml 1% lidocaine 1-2 intercostal spaces below it, down to pleura, draw off 10-30ml pleural fluid with a 21G needle syringe (aspirate while advancing - if suspect problem - CXR after procedure)

Post procedure

• Samples are needed for the following:
• ABG machine pH <7.2 suggests pleural infection: parapneumonic effusion, empyema, malignancy)
• Biochemistry for - protein, glucose, pH, LDH, amylase

• A - sit the patient up
• B - 15L O2 NRBM
• C - Slow drainage - pleural tap/intercostal drain - aim for <2L/24h
• Treat once the underlying cause is established

• USS guided pleural tap or narrow bore pig tailed catheter/formal intercostal chest drain
• Indications - pleural fluid analysis is inconclusive and biopsy needed, drain required for empyema, aim for 1L/h to prevent risk of re-expansion pulmonary oedema - CXR post procedure to check lung re-expansion

• Chemical pleurodesis - with tetracycline/bleomycin/talc
• Indications - Malignant/recurrent effusions (thorascopic talc pleurodesis most effective for malignant effusions) - done via chest drain

• Long term pleural drain - if unfit for surgery, terminal illness but when repeated hospital admission can be avoided
• Surgical - diagnosis or therapy for malignant effusions, persistent collections, increased pleural thickness on USS
• Surgical pleurodesis - VATS (video assisted thorascopic surgery) - camera inserted into pleural cavity with patient laid lateral, fluid sent for cytology, biopsies taken - once histological diagnosis of malignancy is confirmed, pleurodesis performed
• Pleuro-peritoneal shunt
• Indications - if tumour deposits prevent lung contacting chest wall (pleurodesis will fail)
• Shunt from pleural to peritoneal cavity - one way pump inserted to allow drainage of pleural space

• Recurrence - after drainage - reduced risk if there is concomitant pleurodesis, lung collapse, empyema, pneumothorax
• Prognosis is depended on the underlying disease

• All patients with a pleural effusion in association with sepsis or a pneumonic illness require diagnostic pleural fluid sampling
• If the fluid is purulent or turbid/cloudy a chest tube should be placed to allow the drainage
• If the fluid is clear but the pH is <7.2 in patients with suspected pleural infection, a chest tube should be placed

• Any group of carcinomas of lung - arises from the epithelium of the bronchial tree

• Cigarette smoking
• Asbestos
• Chromium
• Arsenic
• Iron oxides
• Radiation (radon gas)

• 19% of all cancers
• 40,000/yr in UK
• F>M

• Non-small cell
• Squamous 75% smokers, central
• Adenocarcinoma 25% non-smokers, peripheral
• Large cell 10%
• Alveolar cell <1%
• Small cell lung cancer
• Also called oat cell 25%
• Other lung tumours
• Bronchial adenoma (rare, slow growing) - carcinoid 90%, cylindroma 10%
• Harmartoma - rare, benign
• Mesothelioma

• Cough 80%
• Haemoptysis 70%
• SOB 60%
• Chest pain 40%
• Recurrent slowly resolving pneumonia
• Anorexia
• Weight loss
• Pressure symptoms - dysphagia (mediastinal lymphadenopathy)

Signs

• Cachexia
• Anaemia
• Clubbing
• HPOA (wrist pain - squamous)
• Lymphadenopathy (supraclavicular/axillary)
• Consolidation/collapse/pleural effusion

Metastases

• Bone tenderness
• Hepatomegaly
• Confusion
• Fits
• Focal CNS signs
• Cerebellar syndrome
• DANISH - dysdiadochokinesia, ataxia, nystagmus, intention tremor, slurred speech, hypotonia
• Proximal myopathy
• Peripheral neuropathy
• Dermatomyositis
• Acanthosis nigricans
• Thrombophlebitis migrans

Causes of a CXR nodule (MEGA CF CHAVS)

• Malignancy
• Encysted effusion - fluid/blood/gas
• Granuloma
• Abscess
• Cyst
• Foreign body
• Carcinoid tumour
• Pulmonary hamartoma
• AV malformation
• Skin tumour e.g. seborrhoeic wart

• FBC - pre-chemo therapy
• INR - pre-biopsy
• U and E’s - pre-chemo therapy
• Calcium
• LFTs

• Chest x-ray
• Peripheral circular opacity (nodule), hilar enlargement, consolidation, lung collapse, pleural effusion, bony secondaries
• Contrast enhanced CT
• For staging - often shows malignancy as speculated (and >2cm - as benign nodules usually <1cm if malignancy not suspected from this may repeat at 3 months (otherwise get PET-CT)
• Chest, liver, adrenal glands
• PET-CT
• To exclude occult metastases which show as hot spots - active cancers cells take up the glucose in the radioactive tracer - especially in the lung bases

• Sputum for MC and S, cytology
• USS guided pleural tap - cytology send at least 20ml
• CT guided FNA/biopsy - once CT with contrast and PET-CT is done for lymph nodes or if primary is located peripherally - risk of pneumothorax so should not fly for 6 weeks after
• Bronchoscopy - if primary located centrally
• Lung function tests

• TNM staging
• T - primary tumour
• Tx (malignant cells in bronchial secretions/nowhere else)
• Tis (Ca-in-situ)
• T0 (none evident)
• T1 (<3cm, lobar or distally)
• T2 (> 3cm and >2cm distal to carina, any size if pleura involved)
• T3 (local structures – chest wall, diaphragm, mediastinal pleura, pericardium – but <2cm from carina)
• T4 (involves carina, or malignant effusion present)
• N - regional nodes
• N0 (none involved)
• N1 (peribronchial and/or ipsialteral hilar)
• N2 (ipsilateral mediastinal/subcarinal)
• N3 (contralateral mediastinum, hilum, scalene/supraclavicular)
• M - distant metastases
• M0 (none)
• M1 (present)

• occult: Tx N0 M0
• I: Tis-2 N0 M0
• II: T1-2 N1 M0 or T3 N0 M0
• IIIa: T3 N1 M0 or T1-3 N2 M0
• IIIb: T1-4 N3 M0 or T4 N0-2 M0
• IV: T1-4 N0-3 M1

• Analgesia
• Anti-emetics
• Steroids
• Cough linctus (codeine)
• Bronchodilators
• Anti-depressants

• Surgical exision - if patient has reasonable performance status - for peripheral tumours with no mets (stage I/II: 25%)
• Radiotherapy - if poor respiratory reserve

• Chemotherapy +/- radiotherapy for advanced disease

• Usually relapse
• Chemotherapy regimes - cyclophosphamide/doxorubicin/vincristine/etoposide or cisplatin +/- radiotherapy if limited disease

• Radiotherapy - bronchial/SVC obstruction (and SVC stent/dexamethasone, hemoptysis, bone pain, cerebral mets
• Endobronchial therapy - tracheal stenting, cryotherapy, laser brachytherapy
• Pleural drainage +/- pleurodesis for symptomatic pleural effusions

• Local ca invasion - recurrent laryngeal/phrenic nerve palsies, SVCO, Horner’s syndrome (Pancoast tumour), rib erosion, pericarditis, AF
• Metastatic - brain, bone (pain, anaemia, hypercalcaemia), liver, adrenal (Addisons)
• Endocrine - ectopic hormone secretion
• SCLC - SIADH, hyponatraemia, increased ADH may be caused by atypical pneumonias
• Cushing’s: increased ACTH, squamous - increased PTH and PTH related peptide/hypercalcaemia
• Non-metastatic neurological - as above
• Lambert-Eaton syndrome (from SCLC - similar symptoms as Myasthenia Gravis) -

• 50% 2 year survival without spread (10% with spread)

• Median survival 3 months (untreated)

* 1-1.5 years treated

• Accounts for 27% of cancer deaths

• Smoking cessation

* Prevent occupational exposure to carcinogens

• Secondary lung tumours are metastatic deposits from primary cancers originating outside the lung

• Genitourinary
• Germ cell tumour
• Bladder
• Prostate
• Renal cell (or Wilm’s tumour in children)
• Gynaecological
• Cervix
• Ovary
• Breast
• Gastrointestinal
• Coloractal
• Skin
• Melanoma
• Bone
• Osteosarcoma
• Endocrine
• Thyroid

• More common than primary lung cancer

* Epidiemiological characteristics reflect that of the primary cancer

• Nearly always in the parenchyma (having spread from the primary site from via the blood/lymphatics)

• Asymptomatic - often even with positive chest x-ray findings
• Occasionally - pleuritic pain, cough, haemoptysis (bronchial involvement), progressive SOB (only if extensive or lymphangitis carcinomatosa)

• Lymphangitis carcinomatosa (when carcinoma – stomach/pancreas/breast – involves mediastinal glands and spreads along the lymphatics of both lungs)
• Primary bronchial Ca
• Tuberculoma
• Benign tumour of lung
• Hydatid cyst

• Chest x-ray
• Discrete round shadows 1.5-3cm wide
• Solitary (cannon-ball mets) usually reflects renal cell Ca, but may also be choriocarcinoma (lymphangitis carcinomatosa has BHL with streaky basal shadowing fanning out over both lungs)
• CT scan
• Same findings but may show more mets

• Medical - chemotherapy may be suitable for patients with sub groups of chemo-responsive tumours e.g. breast Ca
• Majority of patients are palliative
• Surgical pulmonary mastectomy - rarely done as CT usually shows more mets than CXR
• Indications: limited pulmonary mets, where primary tumour is controlled (no evidence of local recurrence or extrapulmonary mets), able to tolerate lung resection

• Onset of pulmonary mets indicates end-stage disease, 50% of resected tumours recur at a median of 10mths

• Operative mortality of pulmonary metastasectomy 1% (5- and 10y survival is 36% and 26%)

• Multisystem disorder of unknown aetiology characterised by non-caseating granulomas
• More common in adults and people of African decent

• Acute - erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthroparthralgia
• Insidious - dyspnoea, non-productive cough, malaise, weight loss,
• Skin - lupus pernio - pathognomic
• Hypercalcaemia - macrophages inside the granulomas cause an increase in the conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

• Grading

0 - Normal
1 - Bilateral hilar lyphadenopathy (BHL present in 65% cases, resolve in 80%)
2 - BHL + peripheral pulmonary infiltrate (22% of cases, 50% resolution)
3 - Peripheral pulmonary infiltrate alone without BHL (13% cases, 25% resolution)
4 - Progressive pulmonary fibrosis

• Unknown but associations with
• Dust inhalation
• Genetic (increased in monozygotic twins)
• HLA-B8 especially with arthritis and erythema nodosum, EN
• HLA-DRB1
• HLA-DBQ1

• 5/100,000/yr in the UK
• F>M
• Onset 20-40 years
• 15 x more common/severe in Afro-Carribeans especially extra-thoracic
• Intra-thoracic involvement most common
• Acute presentation most common

• Granuloma - non-caseating granulomas in affected organs (skin, eyes, respiratory tract)
• Lung - Lymphocytic alveolitis - T lymphocyte stimulated recruitment of macrophages organise into granulomas and mediate inflammation/long term damage

• EN +/- polyarthralgia
• BHL
• No fibrosis

• No EN
• No BHL
• Pulmonary fibrosis with slowly progressive SOB

• Asymptomatic - 20-40% routine chest x-ray
• Pulmonary - 90% BHL +/- infiltrates/fibrosis, pleural effusion (<5%), dry cough, progressive SOB, reduced exercise tolerance, chest pain, symptoms progress in 10-20% leading to reduced lung function

• Lymphadenopathy - 25-80%
• Cervical>axillary>inguinal
• Skin - 10-25%
• EN, lupus pernio (purplish indurated nodule), SC nodules
• Splenomegaly - 10-25%
• Palpable
• Arthritis
• Mainly hands, feet (terminal phalangeal bone cysts), occasionally large joints (Lofgren’s syndrome - BHL + arthritis/arthralgia + erythema nodosum + fever
• Hypercalcaemia/hypercaluria 10%
• Especially in summer - renal calculi, nephrocalcinosis, eyes 3-12%: early anterior uveitis, late posterior uveitis - blindness, conjunctivitis, keratoconjunctivitis sicca (Heerfordt’s sydrome - uveitis + parotid enlargement + cranial nerve palsies + subacute meningitis + systemic symptoms
• CNS - 5%
• Chronic meningitis, hypothalamic lesions (Bell’s palsy), seizures, peripheral/cranial neuropathy
• Others
• Parotid/lacrimal gland enlargement (<5%), URT (6%), liver (70% have granuloma on biopsy), heart (10-20% abnormal ECG: heart block, VT, HF)

• Causes of BHL
• Sarcoidosis
• Infection (TB, mycoplasma)
• Malignancy (lymphoma, Ca, mediastinal tumours)
• Organic dust disease (silicosis, berylliosis - from fluorescent light tubes)
• Pnemoconioses, extrinsic allergic alveolitis, histiocytosis X, metastatic lung disease

• Causes of granuloma
• Infection (bacterial: TB/leprosy/syphilis/Cat scratch fever; fungi: Cryptococcus neoformans; protozoa: Schistosomiasis),
• Autoimmune (PBC, granulomatous orchitis)
• Vasculitis (giant cell arteritis, PAN, Takayasu’s, Wegener’s granulomatosis)
• Organic dust disease (silicosis, berylliosis),
• Idiopathic (Crohns, de Quervain’s thyroiditis, sarcoidosis)
• Extrinsic allergic alveolitis, Histocytosis X (i.e. Langerhan’s cell histiocytosis)

• Causes of increased ACE
• Hyperthyroidism
• TB
• Asbestosis
• Pneumocytosis
• Hypersensitivity pneumonitis
• Gaucher’s silicosis

• FBC - low WCC esp lymphocytes in active, low platelets
• ESR - raised
• U and E’s - raised Ca2+ especially in summer
• LFT’s - Ig’s,
• Serum ACE
• Increased CSF - ACE may diagnose CNS sarcoidosis when serum ACE negative
• 2 ASO titres (to diagnoses EN, along with tuberculin skin test)

• 24h collection - raised Ca2+ especially in summer

• Abnormal chest x-ray in 90%
• BHL
• Infiltrates/fibrosis
• Bulla formation (honey combing)
• Pleural effusion (<5%)
• Bone x-ray
• Punch out lesions in terminal phalanges
• USS
• Nephrocalcinosis
• Hepatosplenomegaly
• High resolution CT/MRI
• To assess severity of pulmonary disease/diagnose neurosarcoidosis

• Tissue biopsy
• Diagnostic for non-caseating granulomata e.g. lung transbronchial +ve in 80%
• Lung function tests
• May be normal (if fibrosis: ↓lung volumes, impaired gas transfer, restrictive ventilatory defect)
• BAL: ↑lymphocytes (active disease), ↑neutrophils (pulmonary fibrosis)
• Tuberculin skin test: -ve in 2/3 cases (+ve may indicate EN)
• ECG: may show arrhythmias/BBB
• Ophthalmology assessment (if ocular disease): slit lamp, fluorescein angiography; Kvein test: now obsolete

• Bed rest

* NSAIDs (most patients with BHL/EN recover spontaneously)

• Indications
• Parenchymal disease
• Uveitis
• Hypercalcaemia - macrophage inside granuloma secretes calcium
• Neuro or cardiac involvement
• Types
• Prednisolone 40mg/24h po for 4-6 weeks - reduce dose over 1 year
• For symptoms
• Topical steroids for uveitis
• Methylprednisolone for severe illness
• Methylprednisolone IV (if severe)
• Others if severe
• Immunosuppressants (methotrexate, hydroxychloroquine - especially in cutaneous/progressive pulmonary disease, ciclosporin, cyclophosamide, anti-TNFa

• Lung transplant if severe

• Generally 60% thoracic sarcoidosis resolve in 2 years (20% respond to steroids) - worse if older age, more widespread disease or Afro-Carribean
• Acute - 80% spontaneous resolution in 1 years
• Chronic - poor
• Associated with progression to pulmonary fibrosis

• <3%

• Patients with pulmonary disease and radiological changes persisting for > 6mths have better long-term outcome if given oral steroids (prednsiolone 30-40mg/24h po) for 6mths

• Acute form of sarcoidosis
• Characterised by BHL, erythema nodosum, fever, and polyarthralgia
• Usually has an excellent prognosis

• Uveoparotid fever
• Parotid enlargement
• Fever and uveitis secondary to sarcoidosis

• Autosomal recessive disorder
• Causing increased viscosity of secretions e.g. lungs and pancreas
• Due to defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR)
• Codes cAMP regulated chloride channel

• 80% of causes in the UK are due to delta F508 on the long arm of chromosome 7

• Affects 1 in 2500 births

* Carrier rate is 1 in 25

• Staphylococcus aureus
• Pseudomonas aeruginosa
• Burkholderia cepacia
• Aspergillus

• Abnormal ion transport
• Defective Cl secretions/increased sodium absorption across epithelial cells of exocrine glands of respiratory tract/pancreas, leads to increased viscosity of secretions
• Abnormal sweat gland functions
• Increased Na+/Cl- in sweat (60-125mmol/L vs 10-30 normally)
• Infection
• Abnormal CFTR also affects the inflammatory processes/defence against infections in lungs
• Mucus in smaller airways predisposes to chronic infection
• Initially with s. aureus, h. influenza, or strep. pneumoniae in young patients
• Later with pseudomonas aeruginosa or burkholderia with rapid progression of lung disease and bronchial wall damage

• Diagnosed on screening

• Recurrent/persistent chest infections
• Prolonged neonatal jaundice
• Meconium ileus 10-20% fail to pass in 48h - rx with gastrografin enemas, most require surgery, malabsorption >90% pancreatic insufficiency, steatorrhoea

• Cough
• Wheeze
• Recurrent infections
• Bronchiectasis (persistent, loose cough, sputum)
• Rectal prolapse
• Nasal polyps
• Sinusitis

• Bronchiectasis
• Pneumothorax
• Recurrent haemoptysis
• Respiratory failure
• Cor pulmonale
• DBM
• Steatorrhoea
• Cirrhosis/portal HTN
• Gallstones
• Distal intestinal obstruction
• Male sterility
• Psychological problems

• Clubbing - established disease
• Short stature
• Chest hyperinflation
• Bilateral coarse inspiratory crackles and/or expiratory wheeze
• Growth chart in children
• Crossing centile lines (height disproportionately greater than weight)

• Dehydration
• Malnutrition
• Atopic eczema
• Hypothyroidism
• Adrenal insufficiency
• Ectodermal dysplasia
• Glycogen storage diseases

• Oedema

* Poor technique

• DNA karyotyping
• Immuno-reactive trypsin - raised in neonates - used in the Guthrie newborn screening
• Aspergillous serology
• PCR (for Burkholderia cepacia)
• FBC
• U and E
• LFT’s
• Vitamin levels A/D/E/K
• Annual OGTT

• Chest x-ray
• Signs of consolidation
• Bronchiectasis
• Cystic changes with chronic pseudomonas infection
• Abdominal USS
• Fatty liver
• Cirrhosis
• Chronic pancreatitis

• Sweat test - diagnostic
• Minimum 100ml needed by pilocarpine iontophoresis onto filter paper/tube
• Na+ >60-125mmol/L (vs normal 10-30) and Cl raised often >Na
• Stool
• Reduced faecal chymotrypsin/elastase
• Faecal fat concentrations (used to monitor pancreatic enzyme supplementation)
• Lung function tests
• Obstructive pattern
• Sputum - MC and S
• Aspergillus skin test - 20% develop ABPA

• Genetic counselling
• MDT: respiratory physician
• PT (3x daily: postural drainage, active cycle breathing/forced expiratory techniques), dietician, specialist nurses, psychosocial support
• Home Rx: whenever possible (parents/patient taught chest PT – regularly performed to clear secretions)
• Pulmonary rehab (for advanced): O2, diuretics, NIV, transplant (see below); - Patient isolation: if Burkholderia cepacia identified on PCR

• Prophylactically (to prevent chest infections: e.g. nebulised ticarcillin/tobramycin at home)
• Targeted during acute infective exacerbation → ticarcillin 80mg/kg (max. 3.2g)/4-8h IV (if > 1y old) plus gentamicin or ceftazidine 50mg/kg/8h IV (panresistant Pseudomonas aeruginosa often needs regular courses of Colistin and meropenem IV/nebs Abx → often given at home via indwelling central venous catheter e.g. Portacath)

• Mucolytics: Dornase alpha (a DNase) 2.5mg/24 nebs
• Bronchodilators: inhaled salbutamol (if reversible obstruction)
• Pancreatic enzyme capsules (Pancrease/Creon to aid meal absorption): contain lipase, amylase, protease
• Dietary supplements (for sufficient caloric intake as energy requirements may be 140% normal during recurrent infection/cough/malabsorption): fat-soluble vitamins (A/D/E/K), ursodeoxycholic acid (for impaired liver function)
• Others: Rx DM, screen for/Rx osteoporosis, arthritis, sinusitis, vasculitis, fertility/genetic counselling

• Heart - lung transplant
• Indications
• When progress leads to end stage
• (FEV1 <30% despite max. therapy – provided good nutrition and no Aspergillus/TB)
• Heart from the CF patient can be used in another recipient (domino transplant)
• Liver transplant for cirrhosis

• Risk of haemoptysis, pneumonia, pneumothorax, HPOA, respiratory failure, DBM, cirrhosis, cholesterol, gall stones, fibrosing colonopathy, male infertility

• No current cure - worse with infection of Burkholderia cepacia infection
• Median survival 40 years (95% die from respiratory failure)

• Full vaccinations (plus pneumococcal)

• Aspiration of a FB can lead to complete upper airway obstruction - this is a medical emergency

• Child
• Toys
• Food (peanuts)
• Adult
• Reduced GCS
• Head injury
• CVA
• OD,
• Sedation
• Anaesthesia
• Reduced coughing reflexes
• Bulbar dysfunction
• Intubation/extubation
• Guillain-Barre syndrome
• MS
• Myasthenia gravis
• Tendency to regurgitate/vomit - Alcohol, full stomach, upper GI, laryngeal/oesophageal pouch, hiatus hernia, oesophageal obstruction

• 80% in <4y old, delayed presentation in 30% - days/weeks (cough, wheeze, haemoptysis, unresolved pneumonia, abscess, empyema)
• Right lower lobe most often affected

• Cough (sudden onset and persistent - larynx/tracheobronchial tree)
• Gagging
• Choking
• Stridor
• Unable to speak

HPC - recent playing with toys, eating, vomiting, regurgitation

PMH - CVA/TIA, hiatus hernia, reflux disease

• Respiratory distress
• Increased HR, RR
• Cyanosis
• Nasal flaring (mouth breathing if 1 yr)
• Grunting
• Tracheal tug
• Use of accessory muscles (head bobbing in infant)
• Subcostal recession (infants)
• Audible sounds (stridor, wheeze, problems talking/drinking/speaking)
• Reduced GCS
• LOC
• Death
• On auscultation
• Often normal but may reveal wheeze/localised absence of breath sounds

• Epiglottitis

* Bacterial tracheitis

• ABG - low PaO2, low PaCO2 - initially from hyperventilation, then increases
• Expiratory chest x-ray
• > 90% show radio-opaque FB with distal atelectasis and consolidation/hyperinflation (FB acting as a ball valve - if severe - diffuse bilateral infiltrates and pulmonary oedema

• BLS choking algorithm

Airway

• Effective cough - fully responsive, loud cough, able to take breath
• Encourage coughing, continue to check for deterioration/ineffective cough or relief of obstruction
• Ineffective cough
• Unconscious - open airway, 5 rescue breaths, CPR at 15:2 for children, if profoundly hypoxic get surgical airway
• <12 yrs Needle cricothyroidectomy
• > 12 yrs Surgical cricothyroidectomy
• Conscious - 5 back blows, then 5 thrusts (chest if <1yr - head down in prone position, abdominal if >1 yr - head down and forward leaning position - repeat up to 5 times

Breathing

• 15L/min O2 NRBM to correct hypoxia
• Nebulised salbutamol 2.5-5mg (for bronchospasm)
• Surgical - referral to cardiothoracic surgeon for urgent bronchoscopy

• Protect airway
• Suction oropharynx (Yanker catheter - avoiding stimulation of gag reflex)
• Tracheal intubation (if necessary)
• NG tube (for at risk patients, to decompress the stomach)

• Infection causing a multi-system caseating granulomatous inflammation
• Most commonly affects the lungs

• Typicals
• M. tuberculosis
• M. Bovis
• M. Africanum
• Atypicals (Immuno-compromised/chronic lung disease)
• M. Avium-intracellulare (bacteraemia in AIDS/lymphadenopathy)
• M. Kansasii (pulmonary infection in chronic lung disease
• M. Marinum (fish tank granuloma)
• M. Ulcerans (tropical Buruli ulcer)

• High risk
• Immigrants from countries where TB is common
• Alcoholics
• Household contact of open TB
• Immunocompromised (HIV/elderly/very young)
• Others
• Poorly ventilated housing
• Overcrowding
• Poor nutrition
• Poverty
• Low CD4
• Raised ESR
• Many co-infections
• High viraemia

• 4672 UK incidence 2018

* Most common cause of death among HIV patients

• Primary

* Secondary

• Non-immune host who is exposed to M. tuberculosis may develop primary infection of the lungs
• A small lung lesion known as Ghon focus develops
• Ghon focus is composed of tubercule-laden macrophages
• Combination of a Ghon focus and hilar lymph nodes is known as a Ghon complex
• In immunocompotent people the initially lesion usually heals by fibrosis
• In immunocompromised it may develop disseminated disease (miliary tuberculosis)

• If the host becomes immunocompromised the initial infection may become reactivated more severely
• Reactivation generally occurs in the apex of the lungs and may spread locally or to more distant sites
• Intense caseating granuloma (central caseous necrosis) - abscess (cold, not hot like like other pyogenic infection)
• Possible causes of immunocompromise include:
• Immunosuppressive drugs
• HIV
• Malnutrition
• Lungs remain the most common site for secondary tuberculosis
• Extra-pulmonary infection may occur in the following areas:
• CNS - tuberculosis meningitis - most serious complication
• Vertebral bodies (Pott’s disease)
• Cervical lymph nodes (scrofuloderma)
• Renal
• Gastrointestinal tract

• Asymptomatic (usually)

• Depends on site of reactivation
• Lung - cough, sputum, haemoptysis, pleurisy
• Meninges - headache, neck stiffness, cranial nerve lesions, coma
• Bone - pain, paraplegia if spinal erosion
• Lymph nodes - glandular enlargement, cold abscess
• Genitourinary - frequency, dysuria, loin/back pain, haematuria, sterile pyuria
• Peritoneal TB - abdominal pain
• Systemic features - fever, night sweats, anorexia, weight loss, malaise

• Lung - may be pleural effusion/superimposed pulmonary infection
• Eye - retinal TB (if miliary)
• Skin - jelly like nodules (face/neck)

• Sarcoidosis - may mimic miliary TB

• FBC - rifampicin can cause low platelets
• U and E’s - altered with TB medications
• LFT - raised ALT/AST with anti TB meds
• CRP
• Quanterferon TB Gold (used if Mantoux +ve/unreliable - tests delayed hypersensitivity reaction as gamma inferon produced from lymphocytes against TB Ag used is not in BCG so +ve test cannot be due to previous BCG - but positive test may be due to latent as well as active TB, may not work as well in immunocompromised)
• HIV (predisposes to TB)
• Sputum culture 1
• NAAT test 2
• Sputum smear 3

• EMU - 3 x early morning samples (for TB culture if genitourinary TB suspected)

• Chest x-ray - Must be done in presenting non-respiratory TB to exclude respiratory TB - consolidation, apical opacities with cavitation, fibrosis, calcification, reticulonodular shadowing (if miliary TB)
• Renal USS - If genitourinary TB is suspected

• Chest x-ray
• Sputum smear
• Sputum culture - gold standard 1-3 weeks to grow
• NAAT

• Main technique used to screen for latent TB
• In recent years the interferon-gamma test has been used
• Used in a number of situations
• Mantoux test is positive or equivocal
• People where a tuberculin test may be falsely negative

• 0.1ml of 1:1000 purified derivative (PPD) injected intradermally
• Result is read 2-3 days later

• <6mm
• Negative - no significant hypersensitivity to tuberculin protein
• Previously unvaccinated individuals may be given the BCG
• 6-15mm
• Positive - hypersensitive to tuberculin protein
• Should not be given BCG
• May be due to previous TB infection or BCG
• > 15mm
• Strongly positive - strong hypersensitive to tuberculin protein
• Suggests tuberculosis

• Miliary TB
• Sarcoidosis
• HIV
• Lymphoma
• Very young age (e.g. <6 months)

• Macrophages often migrate to regional lymph nodes
• Lung lesion plus affected lymph nodes is referred to the Ghon complex
• This leads to the formation of a granuloma which is a collection of epithelioid histiocytes
• There is the presence of caseous necrosis in the centre
• The inflammatory response is mediated by a type 4 hypersensitivity reaction
• In healthy individuals the disease may be contained, in the immunocompromised disseminated TB may occur

• Initial phase - first 2 months (RIPE)
• Rifampicin - liver disease
• Isoniazid - peripheral neuropathy, agranulocytosis
• Pyrazinamide
• Ethambutol - optic neuritis
• Continuation phase - next 4 months
• Rifampicin
• Isonaizid

• 3 months of isoniazid (with pyridoxine) and rifampicin OR 6 months of isoniazid (with pyridoxine)

• 12 month treatment period with the addition of steroids to the regime

• Homeless people with active TB
• Patients who are likely to have poor concordance
• All prisoners with active or latent TB

• Mechanism of action: inhibits bacterial DNA dependent RNA polymerase preventing transcription of DNA into mRNA
• Potent liver enzyme inducer
• Hepatitis, orange secretions
• Flu-like symptoms

• Mechanism of action: inhibits mycolic acid synthesis
• Peripheral neuropathy: prevent with pyridoxine (Vitamin B6)
• Hepatitis, agranulocytosis
• Liver enzyme inhibitor

• Mechanism of action: converted by pyrazinamidase into pyrazinoic acid which in turn inhibits fatty acid synthase (FAS) I
• Hyperuricaemia causing gout
• Arthralgia, myalgia
• Hepatitis

• Mechanism of action: inhibits the enzyme arabinosyl transferase which polymerizes arabinose into arabinan
• Optic neuritis: check visual acuity before and during treatment
• Dose needs adjusting in patients with renal impairment

• Recurrent and intermittent closure/collapse of pharygeal airway causing apnoeic episodes during sleep
• Terminated by partial arousal
• Resulting in daytime somnolence (sleepiness/drowsiness)

• > 5 episodes of apnoea/h during sleep (each at least 10s)
• Apnoea Hypopnoea Index of >5
• > 15 is considered significant

• Obesity
• Age
• Male gender
• Small jaw
• Large tonsils
• Marfan’s syndrome

• M>F 4% vs 2%

• Complete or partial collapse of pharyngeal airway
• Hypopneoa
• Reduced SpO2
• Brief arousal - allows a few breaths
• Sleep resumes
• Cycle can repeat up to 60-100/h
• Wake up with daytime somnolence

• Excessive daytime somnolence
• Loud snoring
• Poor sleep quality
• Apnoeic episodes during sleep (reported by partner)
• Choking during sleep (reported by patient)
• Mood changes
• Morning headache
• ↓libido/ED
• ↓cognitive performance
• Poor concentration
• Nocturia

• BMI - obese
• HTN
• Small mandible
• Crowded oropharynx
• Large neck >42cm
• Check nasal patency
• Tongue size
• Uvula

• Co-existing
• COPD
• Hypothyroidism
• Marfan’s syndrome
• Acromegaly
• Narcolepsy
• Restless leg syndrome

• Epworth Sleepiness Scale
• Multiple Sleep Latency Test
• Pulse oximetry
• Polysomnography/sleep study

• Quantifies severity of daytime somnolence /24

• Measures the time to fall asleep in a dark room (using EEG criteria)

• Indications if the patient has Epworth score >10, snoring associated with HTN/CVD, uvulopalatoplasty being considered
• Measures SaO2, airflow at nose/mouth, ECG, EMG, chest/abdominal wall movement - if narcolepsy/restless leg syndrome being considered also monitor EEG, REM

• Weight reduction
• Avoid tobacco
• Avoid Alcohol especially at night
• Positional therapy to prevent lying on back e.g. tennis ball sewn into pyjama back

• CPAP via nasal mask
• Indications - AHI >15 + excessive daytime somnolence, impaired cognition, mood disorders, insomnia, CVD
• Procedure - CPAP mask over nose/mouth, ventilator blows air continuously at fixed pressure usually 10cm H2O keeps airway open during sleep, preventing desaturation/arousal
• Problems - nasal bridge pressure sores, claustrophobia, nasal congestion, sneezing, disrupted sleep
• Mandibular advancement splints
• Indications - mild OSA, intolerant to CPAP
• Procedure - personalised splint made by orthodontist, pulling the mandible forward to keep the airway open during sleep

• Indications - relieve pharyngeal obstruction (tonsillectomy, uvulopalatopharyngoplasty, tracheostomy - last resort - only after discussion with a chest physician

• Risk of pulmonary HTN and systemic HTN
• OSA is an independent risk factor, type 2 respiratory failure, impaired cognition, altered mood/personality, increased risk of RTA’s

Includes the following questions:

How likely are you to doze or fall asleep in the following situations, in contrast to just feeling tired?

• Sitting and reading
• Watching television
• Sitting, inactive in a public place (for example at the theatre or in a meeting)
• As a passenger in a car for an hour without a break
• Lying down to rest in the afternoon when circ*mstances permit
• Sitting and talking to someone
• Sitting quietly after lunch without alcohol
• In a car, while stopped for a few minutes in traffic

Each question is answered choosing from one of the following options:

• Would never doze (0 points)
• Slight chance of dozing (1 point)
• Moderate chance of dozing (2 points)
• High chance of dozing (3 points)

A total score greater than 10 indicates abnormal daytime sleepiness:

• Mild (11–14)
• Moderate (15–18)
• Severe (more than 18)