Respiratory Flashcards
What methods can be used for smoking cessation? how are these prescribed?
patients should be offered nicotine replacement therapy (NRT), varenicline or bupropion - NICE state that clinicians should not favour one medication over another
NRT, varenicline or bupropion should normally be prescribed as part of a commitment to stop smoking on or before a particular date (target stop date)
prescription of NRT, varenicline or bupropion should be sufficient to last only until 2 weeks after the target stop date. Normally, this will be after 2 weeks of NRT therapy, and 3-4 weeks for varenicline and bupropion, to allow for the different methods of administration and mode of action. Further prescriptions should be given only to people who have demonstrated that their quit attempt is continuing
if unsuccessful using NRT, varenicline or bupropion, do not offer a repeat prescription within 6 months unless special circumstances have intervened
do not offer NRT, varenicline or bupropion in any combination
What are the adverse effects of NRT?
Nicotine replacement therapy
adverse effects include nausea & vomiting, headaches and flu-like symptoms NICE recommend offering a combination of nicotine patches and another form of NRT (such as gum, inhalator, lozenge or nasal spray) to people who show a high level of dependence on nicotine or who have found single forms of NRT inadequate in the past
What is varenicline? When should this be started?
a nicotinic receptor partial agonist
should be started 1 week before the patients target date to stop
the recommended course of treatment is 12 weeks (but patients should be monitored regularly and treatment only continued if not smoking)
IS varenicline effective? what are the side effects? when is this contraindicated?
has been shown in studies to be more effective than bupropion
nausea is the most common adverse effect. Other common problems include headache, insomnia, abnormal dreams
varenicline should be used with caution in patients with a history of depression or self-harm. There are ongoing studies looking at the risk of suicidal behaviour in patients taking varenicline
contraindicated in pregnancy and breast feeding
What is bupropion? when should this be started? what are the risks (1) and contraindications (4)?
a norepinephrine and dopamine reuptake inhibitor, and nicotinic antagonist
should be started 1 to 2 weeks before the patients target date to stop
small risk of seizures (1 in 1,000)
contraindicated in epilepsy, pregnancy and breast feeding. Having an eating disorder is a relative contraindication
Are pregnant women tested for smoking? who is referred to smoking cessation?
NICE recommended in 2010 that all pregnant women should be tested for smoking using carbon monoxide detectors, partly because ‘some women find it difficult to say that they smoke because the pressure not to smoke during pregnancy is so intense.’. All women who smoke, or have stopped smoking within the last 2 weeks, or those with a CO reading of 7 ppm or above should be referred to NHS Stop Smoking Services.
What can be used for smoking cessation in pregnancy?
Interventions
the first-line interventions in pregnancy should be cognitive behaviour therapy, motivational interviewing or structured self-help and support from NHS Stop Smoking Services the evidence for the use of NRT in pregnancy is mixed but it is often used if the above measures failure. There is no evidence that it affects the child's birthweight. Pregnant women should remove the patches before going to bed as mentioned above, varenicline and bupropion are contraindicated
COPD
-What lifestyle help is offered? (2)
-what vaccinations are offered?
General management
>smoking cessation advice: including offering nicotine replacement therapy, varenicline or bupropion annual influenza vaccination one-off pneumococcal vaccination pulmonary rehabilitation to all people who view themselves as functionally disabled by COPD (usually Medical Research Council [MRC] grade 3 and above)
what is the first line treatment of COPD? what is the next step determined by?
Bronchodilator therapy
a short-acting beta2-agonist (SABA) i.e. salbutamol or short-acting muscarinic antagonist (SAMA) i.e. ipratropium is first-line treatment for patients who remain breathless or have exacerbations despite using short-acting bronchodilators the next step is determined by whether the patient has 'asthmatic features/features suggesting steroid responsiveness'
How do you determine whether a patient had asthmatic/steroid responsive features? (4)
There are a number of criteria NICE suggest to determine whether a patient has asthmatic/steroid responsive features:
any previous, secure diagnosis of asthma or of atopy a higher blood eosinophil count - note that NICE recommend a full blood count for all patients as part of the work-up substantial variation in FEV1 over time (at least 400 ml) substantial diurnal variation in peak expiratory flow (at least 20%)
What is the second line therapy for COPD if there is no asthmatic features?
No asthmatic features/features suggesting steroid responsiveness
add a long-acting beta2-agonist (LABA) i.e. salmeterol + long-acting muscarinic antagonist (LAMA) e.g. tioptropium
if already taking a SAMA, discontinue and switch to a SABA
What is the second line therapy for COPD if there is asthmatic features?
Asthmatic features/features suggesting steroid responsiveness
LABA + inhaled corticosteroid (ICS)
if patients remain breathless or have exacerbations offer triple therapy i.e. LAMA + LABA + ICS
if already taking a SAMA, discontinue and switch to a SABA
NICE recommend the use of combined inhalers where possible
When is oral theophylline used in COPD?
Oral theophylline
NICE only recommends theophylline after trials of short and long-acting bronchodilators or to people who cannot used inhaled therapy the dose should be reduced if macrolide or fluoroquinolone antibiotics are co-prescribed
When are oral prophylactic antibiotics used for COPD? What are prerequisites? what tests are required before prescribing?
Oral prophylactic antibiotic therapy
azithromycin prophylaxis is recommended in select patients patients should not smoke, have optimised standard treatments and continue to have exacerbations other prerequisites include a CT thorax (to exclude bronchiectasis) and sputum culture (to exclude atypical infections and tuberculosis) LFTs and an ECG to exclude QT prolongation should also be done as azithromycin can prolong the QT interval
When are mucolytics considered for COPD?
Mucolytics
should be 'considered' in patients with a chronic productive cough and continued if symptoms improve
When are oral PDE-4 inhibitors used in COPD?
Phosphodiesterase-4 (PDE-4) inhibitors NICE
oral PDE-4 inhibitors such as roflumilast reduce the risk of COPD exacerbations in patients with severe COPD and a history of frequent COPD exacerbations
NICE recommend if:
the disease is severe, defined as a forced expiratory volume in 1 second (FEV1) after a bronchodilator of less than 50% of predicted normal, and
the person has had 2 or more exacerbations in the previous 12 months despite triple inhaled therapy with a long-acting muscarinic antagonist, a long-acting beta-2 agonist and an inhaled corticosteroid
What are the features of cor pulmonale? what treatments can be used?
Cor pulmonale
features include peripheral oedema, raised jugular venous pressure, systolic parasternal heave, loud P2 use a loop diuretic for oedema, consider long-term oxygen therapy ACE-inhibitors, calcium channel blockers and alpha blockers are not recommended by NICE
What 3 factors may improve survival in COPD?
Factors which may improve survival in patients with stable COPD
smoking cessation - the single most important intervention in patients who are still smoking long term oxygen therapy in patients who fit criteria lung volume reduction surgery in selected patients
What 3 bacteria are the most common in COPD exacerbations? what viral causes is common?
bacteria
Haemophilus influenzae (most common cause)
Streptococcus pneumoniae
Moraxella catarrhalis
respiratory viruses
account for around 30% of exacerbations
human rhinovirus is the most important pathogen
What is the treatment of exac CCOPD?
NICE guidelines from 2010 recommend the following:
increase the frequency of bronchodilator use and consider giving via a nebuliser give prednisolone 30 mg daily for 5 days it is common practice for all patients with an exacerbation of COPD to receive antibiotics. NICE do not support this approach. They recommend giving oral antibiotics 'if sputum is purulent or there are clinical signs of pneumonia' the BNF recommends one of the following oral antibiotics first-line: amoxicillin or clarithromycin or doxycycline.
What oxygen therapy do you give oxygen therapy?
Oxygen therapy
COPD patients are at risk of hypercapnia - therefore an initial oxygen saturation target of 88-92% should be used prior to the availability of blood gases, use a 28% Venturi mask at 4 l/min and aim for an oxygen saturation of 88-92% for patients with risk factors for hypercapnia but no prior history of respiratory acidosis adjust target range to 94-98% if the pCO2 is normal
What nebs/steroids or additional therapy would you give for acute COPD?
Nebulised bronchodilator
beta adrenergic agonist: e.g. salbutamol muscarinic antagonists: e.g. ipratropium
Steroid therapy as above
IV hydrocortisone may sometimes be considered instead of oral prednisolone
IV theophylline
may be considered for patients not responding to nebulised bronchodilators
when is NIV used for COPD patients? When is BiPaP used?
Patients with COPD are prone to develop type 2 respiratory failure. If this develops then non-invasive ventilation may be used
typically used for COPD with respiratory acidosis pH 7.25-7.35
the BTS guidelines state that NIV can 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) and a lower threshold for intubation and ventilation should be used
bilevel positive airway pressure (BiPaP) is typically used with initial settings:
Expiratory Positive Airway Pressure (EPAP): 4-5 cm H2O
Inspiratory Positive Airway Pressure (IPAP): RCP advocate 10 cm H20 whilst BTS suggest 12-15 cm H2O
What is A1AT caused by?
Alpha-1 antitrypsin (A1AT) deficiency is a common inherited condition caused by a lack of a protease inhibitor (Pi) normally produced by the liver. The role of A1AT is to protect cells from enzymes such as neutrophil elastase. It classically causes emphysema (i.e. chronic obstructive pulmonary disease) in patients who are young and non-smokers.
Where is A1AT gene located? how is this inherited?
Genetics
located on chromosome 14 inherited in an autosomal recessive / co-dominant fashion*
How are alleles for A1AT classifed?
alleles classified by their electrophoretic mobility - M for normal, S for slow, and Z for very slow
normal: PiMM
heterozygous: PiMZ
evidence base is conflicting re: risk of emphsema
however, if non-smoker low risk of developing emphsema but may pass on A1AT gene to children
homozygous PiSS: 50% normal A1AT levels
homozygous PiZZ: 10% normal A1AT levels
Features A1AT, what genotype do most patients who present have? what is seen in the lungs? what is seen in the liver?
Features
patients who manifest disease usually have PiZZ genotype lungs: panacinar emphysema, most marked in lower lobes liver: cirrhosis and hepatocellular carcinoma in adults, cholestasis in children
What are the investigations for A1AT?
Investigations
A1AT concentrations spirometry: obstructive picture
What is the management of A1AT?
Management
no smoking supportive: bronchodilators, physiotherapy intravenous alpha1-antitrypsin protein concentrates surgery: lung volume reduction surgery, lung transplantation
What are some causes of COPD?
Smoking!
Alpha-1 antitrypsin deficiency
Other causes
cadmium (used in smelting) coal cotton cement grain
what are the investigations for suspected COPD?
The following investigations are recommended in patients with suspected COPD:
post-bronchodilator spirometry to demonstrate airflow obstruction: FEV1/FVC ratio less than 70%
chest x-ray
hyperinflation
bullae: if large, may sometimes mimic a pneumothorax
flat hemidiaphragm
also important to exclude lung cancer
full blood count: exclude secondary polycythaemia
body mass index (BMI) calculation
HOw is COPD classified?
Post-bronchodilator FEV1/FVC FEV1 (of predicted) Severity
< 0.7 > 80% Stage 1 - Mild**
< 0.7 50-79% Stage 2 - Moderate
< 0.7 30-49% Stage 3 - Severe
< 0.7 < 30% Stage 4 - Very severe
Which patients should be assessed for LTOT?
Assess patients if any of the following:
very severe airflow obstruction (FEV1 < 30% predicted). Assessment should be 'considered' for patients with severe airflow obstruction (FEV1 30-49% predicted) cyanosis polycythaemia peripheral oedema raised jugular venous pressure oxygen saturations less than or equal to 92% on room air
How is assessment for LTOT done? who is offered LTOT?
Assessment is done by 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
What is seen in moderate asthma exac.
-PEFR
-Speech
-RR
-Pulse
PEFR 50-75% best or predicted
Speech normal
RR < 25 / min
Pulse < 110 bpm
What is seen in severe asthma exac.
-PEFR
-Speech
-RR
-Pulse
PEFR 33 - 50% best or predicted
Can’t complete sentences
RR > 25/min
Pulse > 110 bpm
What is seen in lifethreatening asthma exac?
PEFR
O2 sats
HR
Chest exam
PEFR < 33% best or predicted
Oxygen sats < 92%
Silent chest, cyanosis or feeble respiratory effort
Bradycardia, dysrhythmia or hypotension
Exhaustion, confusion or coma
n addition, a normal pCO2 in an acute asthma attack indicates exhaustion and should, therefore, be classified as life-threatening.
when is a chest xr indicated in asthma exac?
a chest x-ray is not routinely recommended, unless:
life-threatening asthma suspected pneumothorax failure to respond to treatment
who is offered admission for asthma exac?
admission
all patients with life-threatening should be admitted in hospital patients with features of severe acute asthma should also be admitted if they fail to respond to initial treatment. other admission criteria include a previous near-fatal asthma attack, pregnancy, an attack occurring despite already using oral corticosteroid and presentation at night
How is oxygen therapy managed in asthma exac?
oxygen
if patients are hypoxaemic, it is important to start them on supplemental oxygen therapy if patients are acutely unwell they should be started on 15L of supplemental via a non-rebreathe mask, which can then be titrated down to a flow rate where they are able to maintain a SpO₂ 94-98%.
SABA therapy in asthma
-what is given?
-how is this given?
bronchodilation with short-acting beta₂-agonists (SABA)
high-dose inhaled SABA e.g. salbutamol, terbutaline in patients without features of life-threatening or near-fatal asthma, this can be given by a standard pressurised metered-dose inhaler (pMDI) or by an oxygen-driven nebulizer in patients with features of a life-threatening exacerbation of asthma, nebulised SABA is recommended
What steroids are given in asthma exac?
corticosteroid
all patients should be given 40-50mg of prednisolone orally (PO) daily, which should be continued for at least five days or until the patient recovers from the attack during this time, patients should continue their normal medication routine including inhaled corticosteroids.
When and what SAMA is given in asthma exac?
ipratropium bromide: in patients with severe or life-threatening asthma, or in patients who have not responded to beta₂-agonist and corticosteroid treatment, nebulised ipratropium bromide, a short-acting muscarinic antagonist
When is iv magnesium / iv aminophylline given in asthma exac?
IV magnesium sulphate
the BTS notes that the evidence base is mixed for this treatment that is now commonly given for severe/life-threatening asthma
IV aminophylline may be considered following consultation with senior medical staff
patients who fail to respond require senior critical care support and should be treated in an appropriate ITU/HDU setting. Treatment options include:
intubation and ventilation extracorporeal membrane oxygenation (ECMO)
What is the criteria for discharge of patients after acute exac asthma?
Criteria for discharge
been stable on their discharge medication (i.e. no nebulisers or oxygen) for 12–24 hours inhaler technique checked and recorded PEF >75% of best or predicted
who should get objective tests for asthma diagnosis?
All patients >= 5 years should have objective tests. Once a child with suspected asthma reaches the age of 5 years objective tests should be performed to confirm the diagnosis
What objective tests are used in diagnosing asthma in patients 17 years or older?
Patients >= 17 years
patients should be asked if their symptoms are better on days away from work/during holidays. If so, patients should be referred to a specialist as possible occupational asthma all patients should have spirometry with a bronchodilator reversibility (BDR) test all patients should have a FeNO test
What objective tests are used in the diagnosis of asthma in children between 5-16 years? what if they are younger than 5?
Children 5-16 years
all children should have spirometry with a bronchodilator reversibility (BDR) test a FeNO test should be requested if there is normal spirometry or obstructive spirometry with a negative bronchodilator reversibility (BDR) test
Patients < 5 years
- diagnosis should be made on clinical judgement
When is a FeNO test considered positive?
FeNO
in adults level of >= 40 parts per billion (ppb) is considered positive in children a level of >= 35 parts per billion (ppb) is considered positive
When is spirometry considered obstructive?
Spirometry
FEV1/FVC ratio less than 70% (or below the lower limit of normal if this value is available) is considered obstructive
When is reversibility testing considered positive?
Reversibility testing
in adults, a positive test is indicated by an improvement in FEV1 of 12% or more and increase in volume of 200 ml or more in children, a positive test is indicated by an improvement in FEV1 of 12% or more
What is the first line treatment of asthma?
1
Newly-diagnosed asthma Short-acting beta agonist (SABA)
2
Not controlled on previous step
OR
Newly-diagnosed asthma with symptoms >= 3 / week or night-time waking SABA + low-dose inhaled corticosteroid (ICS)
What is the third line treatment of asthma?
3 SABA + low-dose ICS + leukotriene receptor antagonist (LTRA)
What is the fourth line treatment asthma?
SABA + low-dose ICS + long-acting beta agonist (LABA)
Continue LTRA depending on patient’s response to LTRA
What is the fifth line treatment of asthma?
SABA +/- LTRA
Switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a low-dose ICS
What is the 6th line therapy of asthma?
SABA +/- LTRA + medium-dose ICS MART
OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA
What is the 7th line therapy in asthma?
SABA +/- LTRA + one of the following options:
increase ICS to high-dose (only as part of a fixed-dose regime, not as a MART) a trial of an additional drug (for example, a long-acting muscarinic receptor antagonist or theophylline) seeking advice from a healthcare professional with expertise in asthma
What is maintenance and reliever therapy?
Maintenance and reliever therapy (MART)
a form of combined ICS and LABA treatment in which a single inhaler, containing both ICS and a fast-acting LABA, is used for both daily maintenance therapy and the relief of symptoms as required MART is only available for ICS and LABA combinations in which the LABA has a fast-acting component (for example, formoterol)
what is a low / medium / high dose ICS?
Frustratingly, the definitions of what constitutes a low, moderate or high-dose ICS have also changed. For adults:
<= 400 micrograms budesonide or equivalent = low dose 400 micrograms - 800 micrograms budesonide or equivalent = moderate dose > 800 micrograms budesonide or equivalent= high dose.
Which asthma patients should be referred to secondary care?
Which patients should be invited for urgent review of asthma control?
referral to secondary care should be made if patients have required more than two courses of systemic corticosteroids, oral or injected, in the previous 12 months or require management using British Thoracic Society (BTS) stepwise treatment 4 or 5 to achieve control
all patients who have been prescribed more than 12 reliever inhalers in the past 12 months should be invited for urgent review of their asthma control
Occupational asthma
-What is the diagnosis?
-Who manages these patients?
erial measurements of peak expiratory flow are recommended at work and away from work.
Referral should be made to a respiratory specialist for patients with suspected occupational asthma.
what is correct inhaler technique?
The following inhaler technique guideline is for metered-dose inhalers (source: Asthma.org.uk, a resource recommended to patients by the British Thoracic Society)
- Remove cap and shake
- Breathe out gently
- Put mouthpiece in mouth and as you begin to breathe in, which should be slow and deep, press canister down and continue to inhale steadily and deeply
- Hold breath for 10 seconds, or as long as is comfortable
- For a second dose wait for approximately 30 seconds before repeating steps 1-4.
Only use the device for the number of doses on the label, then start a new inhaler.
what is the management of acute bronchitis?
Management
analgesia
good fluid intake
consider antibiotic therapy if patients:
are systemically very unwell
have pre-existing co-morbidities
have a CRP of 20-100mg/L (offer delayed prescription) or a CRP >100mg/L (offer antibiotics immediately)
NICE Clinical Knowledge Summaries/BNF currently recommend doxycycline first-line
doxycycline cannot be used in children or pregnant women
alternatives include amoxicillin
What are cardiac causes of clubbing?
Cardiac causes
cyanotic congenital heart disease (Fallot's, TGA) bacterial endocarditis atrial myxoma
What are respiratory causes of clubbing?
Respiratory causes
lung cancer pyogenic conditions: cystic fibrosis, bronchiectasis, abscess, empyema tuberculosis asbestosis, mesothelioma fibrosing alveolitis
what are non-cardiac or non-respiratory causes of clubbing?
Other causes
Crohn's, to a lesser extent UC cirrhosis, primary biliary cirrhosis Graves' disease (thyroid acropachy) rare: Whipple's disease
what are three main types of altitude related disorders?
There are three main types of altitude-related disorders: acute mountain sickness (AMS), which may progress to high altitude pulmonary oedema (HAPE) or high altitude cerebral oedema (HACE). All three conditions are due to the chronic hypobaric hypoxia which develops at high altitudes
AMS
-when does this occur
-what are the features?
Acute mountain sickness is generally a self-limiting condition. Features of AMS start to occur above 2,500 - 3,000m, developing gradually over 6-12 hours and potentially last a number of days:
headache nausea fatigue
Describe the prevention and treatent of AMS?
Prevention and treatment of AMS
the risk of AMS may actually be positively correlated to physical fitness
gain altitude at no more than 500 m per day
acetazolamide (a carbonic anhydrase inhibitor) is widely used to prevent AMS and has a supporting evidence base
it causes a primary metabolic acidosis and compensatory respiratory alkalosis which increases respiratory rate and improves oxygenation
treatment: descent
what is the pathophysiology of HAPE?
HAPE
mechanism: hypobaric hypoxia → uneven hypoxic pulmonary vasoconstriction → uneven blood flow in the lungs → areas of the lung receiving more blood experience an increase in capillary pressure → more fluid leakage. Hypoxia may also directly increase capillary permeability, exacerbating fluid leakage into the alveolar space. presents with classical pulmonary oedema features
what is the management of HAPE?
Management of HAPE
descent nifedipine, dexamethasone, acetazolamide, phosphodiesterase type V inhibitors* oxygen if available
What is the pathophysiology of HACE
HACE
in contrast to the above, cerebral vasodilation is the problem. Hypoxia → cerebral vasodilation → elevated cerebral blood volume also, hypoxia → increase in the permeability of the blood-brain barrier → capillaries in the brain more leaky → leading to fluid accumulation in the extracellular spaces both these factors → cerebral oedema presents with headache, ataxia, papilloedema
What is the management of HACE?
Management of HACE
descent dexamethasone
What are pleural plaques cause by asbestosis? do they undergo malignant change?
Pleural plaques are benign and do not undergo malignant change. They, therefore don’t require any follow-up. They are the most common form of asbestos-related lung disease and generally occur after a latent period of 20-40 years.
Asbestosis
-does the severity correlate with exposure?
-what are the features
-what is the management?
The severity of asbestosis is related to the length of exposure. This is in contrast to mesothelioma where even very limited exposure can cause disease. The latent period is typically 15-30 years. Asbestosis typically causes lower lobe fibrosis.
Features
dyspnoea and reduced exercise tolerance clubbing bilateral end-inspiratory crackles lung function tests show a restrictive pattern with reduced gas transfer
It is treated conservatively - no interventions offer a significant benefit.
what is mesothelioma? what are features? what is management?
Mesothelioma is a malignant disease of the pleura. Crocidolite (blue) asbestos is the most dangerous form.
Possible features
progressive shortness-of-breath chest pain pleural effusion
Patients are usually offered palliative chemotherapy and there is also a limited role for surgery and radiotherapy. Unfortunately, the prognosis is very poor, with a median survival from diagnosis of 8-14 months.
What is the most common of cancer assoc with asbestos?
Whilst mesothelioma is in some ways synonymous with asbestos, lung cancer is actually the most common form of cancer associated with asbestos exposure. It also has a synergistic effect with cigarette smoke in terms of the increased risk. Therefore, smoking cessation is very important as the risk of lung cancer in smokers who have a history of asbestos exposure is very high.
What is bronchiectasis? what is the management?
Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation. After assessing for treatable causes (e.g. immune deficiency) management is as follows:
physical training (e.g. inspiratory muscle training) - has a good evidence base for patients with non-cystic fibrosis bronchiectasis postural drainage antibiotics for exacerbations + long-term rotating antibiotics in severe cases bronchodilators in selected cases immunisations surgery in selected cases (e.g. Localised disease)
what are the most common organisms found in bronchiectasis?
Most common organisms isolated from patients with bronchiectasis:
Haemophilus influenzae (most common) Pseudomonas aeruginosa Klebsiella spp. Streptococcus pneumoniae
what is eosinophilic granulomatosis with polyangiitis?
Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis.
What are features of EGPA?
Features
asthma blood eosinophilia (e.g. > 10%) paranasal sinusitis mononeuritis multiplex pANCA positive in 60%
What is granulomatosis with polyangiitis?
Granulomatosis with polyangiitis is now the preferred term for Wegener’s granulomatosis. It is an autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.
What are the 5 features of granulomatosis with polyangiitis?
Features
upper respiratory tract: epistaxis, sinusitis, nasal crusting
lower respiratory tract: dyspnoea, haemoptysis
rapidly progressive glomerulonephritis ('pauci-immune', 80% of patients)
saddle-shape nose deformity
also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
What are the investigations for granulomatosis with polyangiitis?
Investigations
cANCA positive in > 90%, pANCA positive in 25% chest x-ray: wide variety of presentations, including cavitating lesions renal biopsy: epithelial crescents in Bowman's capsule
What is the management of granulomatosis with polyangiitis?
Management
steroids cyclophosphamide (90% response) plasma exchange median survival = 8-9 years
What is EAA?
Extrinsic allergic alveolitis (EAA, also known as hypersensitivity pneumonitis) is a condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles. It is thought to be largely caused by immune-complex mediated tissue damage (type III hypersensitivity) although delayed hypersensitivity (type IV) is also thought to play a role in EAA, especially in the chronic phase.
Give some examples of EAA (4)
Examples
bird fanciers' lung: avian proteins from bird droppings farmers lung: spores of Saccharopolyspora rectivirgula from wet hay (formerly Micropolyspora faeni) malt workers' lung: Aspergillus clavatus mushroom workers' lung: thermophilic actinomycetes*
What is the acute presentation of EAA
Presentation
acute (occurs 4-8 hrs after exposure)
dyspnoea
dry cough
fever
chronic (occurs weeks-months after exposure)
lethargy
dyspnoea
productive cough
anorexia and weight loss
What investigations are found with EAA?
Investigation
imaging: upper/mid-zone fibrosis bronchoalveolar lavage: lymphocytosis serologic assays for specific IgG antibodies blood: NO eosinophilia
What is the management of EAA?
Management
avoid precipitating factors oral glucocorticoids
What is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis (IPF, previously termed cryptogenic fibrosing alveolitis) is a chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs. Whilst there are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos) the term IPF is reserved when no underlying cause exists.
When is IPF seen and what are the 4 features?
IPF is typically seen in patients aged 50-70 years and is twice as common in men.
Features
progressive exertional dyspnoea bibasal fine end-inspiratory crepitations on auscultation dry cough clubbing
what is the diagnosis of idiopathic pulmonary fibrosis?
Diagnosis
spirometry: classically a restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased) impaired gas exchange: reduced transfer factor (TLCO) imaging: bilateral interstitial shadowing (typically small, irregular, peripheral opacities - 'ground-glass' - later progressing to 'honeycombing') may be seen on a chest x-ray but high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF ANA positive in 30%, rheumatoid factor positive in 10% but this does not necessarily mean that the fibrosis is secondary to a connective tissue disease. Titres are usually low
What is the management of idiopathic pulmonary fibrosis? what is the prognosis?
Management
pulmonary rehabilitation very few medications have been shown to give any benefit in IPF. There is some evidence that pirfenidone (an antifibrotic agent) may be useful in selected patients (see NICE guidelines) many patients will require supplementary oxygen and eventually a lung transplant
Prognosis
poor, average life expectancy is around 3-4 years
What is kartageners syndrome?
Kartagener’s syndrome (also known as primary ciliary dyskinesia) was first described in 1933 and most frequently occurs in examinations due to its association with dextrocardia (e.g. ‘quiet heart sounds’, ‘small volume complexes in lateral leads’)
Pathogenesis
dynein arm defect results in immotile cilia
What are the features of kartageners syndrome?
Features
dextrocardia or complete situs inversus bronchiectasis recurrent sinusitis subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes)
What is CRB65?
Assessment in primry care
C Confusion (abbreviated mental test score <= 8/10)
R Respiration rate >= 30/min
B Blood pressure: systolic <= 90 mmHg and/or diastolic <= 60 mmHg
65 Aged >= 65 years
Patients are stratified for risk of death as follows:
0: low risk (less than 1% mortality risk)
NICE recommend that treatment at home should be considered (alongside clinical judgement)
1 or 2: intermediate risk (1-10% mortality risk)
NICE recommend that ' hospital assessment should be considered (particularly for people with a score of 2)'
3 or 4: high risk (more than 10% mortality risk)
NICE recommend urgent admission to hospital
what is the point of care CRP test in pnuemonia?
In primary care
NICE also mention point-of-care CRP test. This is currently not widely available but they make the following recommendation with reference to the use of antibiotic therapy:
CRP < 20 mg/L - do not routinely offer antibiotic therapy CRP 20 - 100 mg/L - consider a delayed antibiotic prescription CRP > 100 mg/L - offer antibiotic therapy
What is curb65?
What is CURB65?
C Confusion (abbreviated mental test score <= 8/10)
U urea > 7 mmol/L
R Respiration rate >= 30/min
B Blood pressure: systolic <= 90 mmHg and/or diastolic <= 60 mmHg
65 Aged >= 65 years
NICE recommend, in conjunction with clinical judgement:
consider home-based care for patients with a CURB65 score of 0 or 1 - low risk (less than 3% mortality risk) consider hospital-based care for patients with a CURB65 score of 2 or more - intermediate risk (3-15% mortality risk) consider intensive care assessment for patients with a CURB65 score of 3 or more - high risk (more than 15% mortality risk)
What are the investigations for pneumonia?
Investigations
chest x-ray in intermediate or high-risk patients NICE recommend blood and sputum cultures, pneumococcal and legionella urinary antigen tests CRP monitoring is recommend for admitted patients to help determine response to treatment
Describe the inpatient management of pneumonia?
Management of low-severity community acquired pneumonia
amoxicillin is first-line if penicillin allergic then use a macrolide or tetracycline NICE now recommend a 5 day course of antibiotics for patients with low severity community acquired pneumonia
Management of moderate and high-severity community acquired pneumonia
dual antibiotic therapy is recommended with amoxicillin and a macrolide a 7-10 day course is recommended NICE recommend considering a beta-lactamase stable penicillin such as co-amoxiclav, ceftriaxone or piperacillin with tazobactam and a macrolide in high-severity community acquired pneumonia
Discharging patients with pneumonia, What would cause a delay in discharge?
NICE recommend that patients are not routinely discharged if in the past 24 hours they have had 2 or more of the following findings:
temperature higher than 37.5°C respiratory rate 24 breaths per minute or more heart rate over 100 beats per minute systolic blood pressure 90 mmHg or less oxygen saturation under 90% on room air abnormal mental status inability to eat without assistance.
They also recommend delaying discharge if the temperature is higher than 37.5°C.
What is the natural history of pneumonia? what is needed as follow up?
NICE recommend that the following information is given to patients with pneumonia in terms of how quickly their symptoms should symptoms should resolve:
Time Progress
1 week Fever should have resolved
4 weeks Chest pain and sputum production should have substantially reduced
6 weeks Cough and breathlessness should have substantially reduced
3 months Most symptoms should have resolved but fatigue may still be present
6 months Most people will feel back to normal.
All cases of pneumonia should have a repeat chest X-ray at 6 weeks after clinical resolution to ensure that the consolidation has resolved and there is no underlying secondary abnormalities (e.g. a lung tumour).
When is an immediate antibiotic prescribing approach considered in primary care for respiratory tract infections?
However, an immediate antibiotic prescribing approach may be considered for:
children younger than 2 years with bilateral acute otitis media children with otorrhoea who have acute otitis media patients with acute sore throat/acute pharyngitis/acute tonsillitis when 3 or more Centor criteria are present
The Centor criteria* are as follows:
presence of tonsillar exudate tender anterior cervical lymphadenopathy or lymphadenitis history of fever absence of cough
and
If the patient is deemed at risk of developing complications, an immediate antibiotic prescribing policy is recommended
are systemically very unwell have symptoms and signs suggestive of serious illness and/or complications (particularly pneumonia, mastoiditis, peritonsillar abscess, peritonsillar cellulitis, intraorbital or intracranial complications) are at high risk of serious complications because of pre-existing comorbidity. This includes patients with significant heart, lung, renal, liver or neuromuscular disease, immunosuppression, cystic fibrosis, and young children who were born prematurely are older than 65 years with acute cough and two or more of the following, or older than 80 years with acute cough and one or more of the following: - hospitalisation in previous year - type 1 or type 2 diabetes - history of congestive heart failure - current use of oral glucocorticoids
How should you advise patients on the length of respiratory tract infections?
The guidelines also suggest that patients should be advised how long respiratory tract infections may last:
acute otitis media: 4 days acute sore throat/acute pharyngitis/acute tonsillitis: 1 week common cold: 1 1/2 weeks acute rhinosinusitis: 2 1/2 weeks acute cough/acute bronchitis: 3 weeks
Haemopytsis with:
History of smoking
Symptoms of malignancy: weight loss, anorexia
What is the diagnosis?
Lung cancer
Haemoptysis with:
Dyspnoea
Bibasal crackles and S3 are the most reliable signs
What is the diagnosis?
pulmonary oedema
Haemoptysis with:
Fever, night sweats, anorexia, weight loss
What is the diagnosis?
TB
Haemoptysis with:
Pleuritic chest pain
Tachycardia, tachypnoea
What is the diagnosis?
Pulmonary embolism
Haemoptysis with:
\Usually acute history of purulent cough
What is the diagnosis?
LRTI
Haemoptysis with:
Usually long history of cough and daily purulent sputum production
what is the diagnosis?
Bronchiectasis
Haemoptysis with:
Dyspnoea
Atrial fibrillation
Malar flush on cheeks
Mid-diastolic murmur
What is the diagnosis
mitral stenosis
Haemoptysis with:
Often past history of tuberculosis.
Haemoptysis may be severe
Chest x-ray shows rounded opacity
What is the diagnosis?
Aspergilloma
Haemoptysis with:
Upper respiratory tract: epistaxis, sinusitis, nasal crusting
Lower respiratory tract: dyspnoea, haemoptysis
Glomerulonephritis
Saddle-shape nose deformity
Granulomatosis with polyangiitis
Haemoptysis with:
Haemoptysis
Systemically unwell: fever, nausea
Glomerulonephritis
Goodpasture’s syndrome
What is a lung abscess? what is the pathophysiology??
A lung abscess is a well-circumscribed infection within the lung parenchyma.
Pathophysiology
most commonly forms secondary to aspiration pneumonia
poor dental hygiene, previous stroke and reduced consciousness are some of the risk factors for this
other potential causes include:
haematogenous spread e.g. secondary to infective endocarditis
direct extension e.g. from an empyema
bronchial obstruction e.g. secondary from a lung tumour
typically polymicrobial
monomicrobial causes include:
Staphylococcus aureus
Klebsiella pneumonia
Pseudomonas aeruginosa
what are 9 features of lung abscess? what are the signs found?
similar features to pneumonia but generally runs a more subacute presentation
symptoms may develop over weeks
systemic features such as night sweats and weight loss may be seen
fever
productive cough
often foul-smelling sputum
haemoptysis in a minority of patients
chest pain
dyspnoea
signs
dull percussion and bronchial breathing
clubbing may be seen
What are the investigations of lung abscess?
chest x-ray
fluid-filled space within an area of consolidation
an air-fluid level is typically seen
sputum and blood cultures should be obtained
What is the management of lung abscess?
Management
intravenous antibiotics if not resolving percutaneous drainage may be required and in very rare cases surgical resection
What is klebsiella pneumoniae? what are the features of klebsiella pneumonia?
Klebsiella pneumoniae is a Gram-negative rod that is part of the normal gut flora. It can cause a number of infections in humans including pneumonia (typically following aspiration) and urinary tract infections.
Features of Klebsiella pneumonia
more common in alcoholic and diabetics may occur following aspiration 'red-currant jelly' sputum often affects upper lobes
What is the prognosis of klebsiella pneumonia
Prognosis
commonly causes lung abscess formation and empyema mortality is 30-50%
What is seen on CXR for lung cancer?
Chest x-ray
this is often the first investigation done in patients with suspected lung cancer in around 10% of patients subsequently diagnosed with lung cancer the chest x-ray was reported as normal
What is the investigation of choice to investigate suspected lung ca? how is tissue biopsy obtained?
CT
is the investigation of choice to investigate suspected lung cancer
Bronchoscopy
this allows a biopsy to be taken to obtain a histological diagnosis sometimes aided by endobronchial ultrasound
Why is pet scanning used in lung cancer?
what is seen on blood tests in lung ca?
PET scanning
is typically done in non-small cell lung cancer to establish eligibility for curative treatment uses 18-fluorodeoxygenase which is preferentially taken up by neoplastic tissue has been shown to improve diagnostic sensitivity of both local and distant metastasis spread in non-small cell lung cancer
Bloods
raised platelets may be seen
Describe the paraneoplastic features of small cell lung ca
Small cell
ADH ACTH - not typical, hypertension, hyperglycaemia, hypokalaemia, alkalosis and muscle weakness are more common than buffalo hump etc Lambert-Eaton syndrome
Describe the paraneoplastic features of squamous cell lung ca
Squamous cell
parathyroid hormone-related protein (PTH-rp) secretion causing hypercalcaemia clubbing hypertrophic pulmonary osteoarthropathy (HPOA) hyperthyroidism due to ectopic TSH
Describe the paraneoplastic features of adenocarcinoma
Adenocarcinoma
gynaecomastia hypertrophic pulmonary osteoarthropathy (HPOA)
What warrants an USOC for lung ca?
Refer people using a suspected cancer pathway referral (for an appointment within 2 weeks) for lung cancer if they:
have chest x-ray findings that suggest lung cancer are aged 40 and over with unexplained haemoptysis
What symptoms warrant urgent CXR for lung ca?
Offer an urgent chest x-ray (to be performed within 2 weeks) to assess for lung cancer in people aged 40 and over if they have 2 or more of the following unexplained symptoms, or if they have ever smoked and have 1 or more of the following unexplained symptoms:
cough fatigue shortness of breath chest pain weight loss appetite loss
What signs warrant urgent CXR for lung ca?
Consider an urgent chest x-ray (to be performed within 2 weeks) to assess for lung cancer in people aged 40 and over with any of the following:
persistent or recurrent chest infection finger clubbing supraclavicular lymphadenopathy or persistent cervical lymphadenopathy chest signs consistent with lung cancer thrombocytosis
Describe obstructive picture PFTs
FEV1 - significantly reduced
FVC - reduced or normal
FEV1% (FEV1/FVC) - reduced
give 4 examples of obstructive lung disease
Asthma
COPD
Bronchiectasis
Bronchiolitis obliterans
describe restrictive picture PFTs
FEV1 - reduced
FVC - significantly reduced
FEV1% (FEV1/FVC) - normal or increased
give 8 examples of restrictive lung disease
Pulmonary fibrosis
Asbestosis
Sarcoidosis
Acute respiratory distress syndrome
Infant respiratory distress syndrome
Kyphoscoliosis e.g. ankylosing spondylitis
Neuromuscular disorders
Severe obesity
What are 4 predisposing factors for OSA?
Predisposing factors
obesity macroglossia: acromegaly, hypothyroidism, amyloidosis large tonsils Marfan's syndrome
What are the clinical features 3 of OSA?
Consequence
daytime somnolence compensated respiratory acidosis hypertension
What assessments and diagnostic tests can be used for sleep apnoea?
Assessment of sleepiness
Epworth Sleepiness Scale - questionnaire completed by patient +/- partner Multiple Sleep Latency Test (MSLT) - measures the time to fall asleep in a dark room (using EEG criteria)
Diagnostic tests
sleep studies (polysomnography) - ranging from monitoring of pulse oximetry at night to full polysomnography where a wide variety of physiological factors are measured including EEG, respiratory airflow, thoraco-abdominal movement, snoring and pulse oximetry
what is the management of obstructive sleep apnoea?
Management
weight loss continuous positive airway pressure (CPAP) is first line for moderate or severe OSAHS intra-oral devices (e.g. mandibular advancement) may be used if CPAP is not tolerated or for patients with mild OSAHS where there is no daytime sleepiness the DVLA should be informed if OSAHS is causing excessive daytime sleepiness limited evidence to support use of pharmacological agents
What are 8 respiratory problems seen in rheumatoid arthritis?
variety of respiratory problems may be seen in patients with rheumatoid arthritis:
pulmonary fibrosis pleural effusion pulmonary nodules bronchiolitis obliterans complications of drug therapy e.g. methotrexate pneumonitis pleurisy Caplan's syndrome - massive fibrotic nodules with occupational coal dust exposure infection (possibly atypical) secondary to immunosuppression
What is sarcoidosis and what are the features? acute vs insidious vs skin vs blood tests?
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent
Features
acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
insidious: dyspnoea, non-productive cough, malaise, weight loss
skin: lupus pernio
hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
what are three syndromes assoc with sarcoidosis?
Lofgren’s syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
In Mikulicz syndrome* there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma
Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
What is silicosis and what is this a risk factor for? which occupations are at risk?
Silicosis is a fibrosing lung disease caused by the inhalation of fine particles of crystalline silicon dioxide (silica). It is a risk factor for developing tuberculosis (silica is toxic to macrophages).
Occupations at risk of silicosis
mining slate works foundries potteries
What are the features of silicosis?
Features
upper zone fibrosing lung disease 'egg-shell' calcification of the hilar lymph nodes
Describe the management primary pneumothorax
Recommendations include:
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 > 2 cm or still short of breath) then a chest drain should be inserted
Describe the management of secondary pnuemothorax
Recommendations include:
if the patient is > 50 years old and the rim of air is > 2cm and/or the patient is short of breath 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 then 1cm) a chest drain should be inserted. All patients should be admitted for at least 24 hours if the pneumothorax is less the 1cm then the BTS guidelines suggest giving oxygen and admitting for 24 hours
What is done for persistent pneumothorax?
If a patient has a persistent air leak or insufficient lung reexpansion despite chest drain insertion, or the patient has recurrent pneumothoraces, then video-assisted thoracoscopic surgery (VATS) should be considered to allow for mechanical/chemical pleurodesis +/- bullectomy
Describe the flying regulations for pneumothorax
Fitness to fly
absolute contraindication, the CAA suggest patients may travel 2 weeks after successful drainage if there is no residual air. The British Thoracic Society used to recommend not travelling by air for a period of 6 weeks but this has now been changed to 1 week post check x-ray
What is the advice for scuba diving if patient has had pneumothorax?
Scuba diving
the BTS guidelines state: 'Diving should be permanently avoided unless the patient has undergone bilateral surgical pleurectomy and has normal lung function and chest CT scan postoperatively.
