Respiratory Flashcards

1
Q

Lung cancer is the third most common cancer in the UK behind breast and prostate. Cigarette smoking is the biggest cause, around what % of lung cancers are thought to be preventable ?

A

Around 80%

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2
Q

What’re the three most common types of lung cancer ?

A
  • Non-small cell lung cancer:
    • Squamous cell carcinoma
    • Adenocarcinoma

-Small cell lung cancer (SCLC)

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3
Q

How can SCLC be responsible for multiple paraneoplastic syndromes ?

A

They contain neurosecretory granules that can release neuroendocrine hormones.

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4
Q

S+S of lung cancer (7) ?

A
  • SOB
  • Cough
  • Haemoptysis (coughing up blood)
  • Finger clubbing
  • Recurrent pneumonia
  • Weight loss
  • Lympadenopathy - often supraclavicular nodes are the first to be found on examination
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5
Q

What is the first line investigation in suspected lung cancer ? + Name four findings that could suggest cancer ?

A

-CXR

  • Hilar enlargement
  • Peripheral opacity - a visible lesion in the lung field
  • Pleural effusion - usually unilateral in cancer
  • Collapse
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6
Q

Besides a CXR what other investigations could be used to investigate for lung cancer (name three/four) ?

A
  • Staging CT chest of chest, abdomen and pelvis
  • PET-CT
  • Bronchoscopy with endobronchial ultrasound (EBUS)
  • Histological diagnosis
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7
Q

Why is CT scan of the chest, abdomen and pelvis done when investigating lung cancer?

A

To establish the stage and check for lymph node involvement and metastasis. This should be contrast enhanced using an injected contrast to give more detailed information about different tissues

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8
Q

What is a PET-CT (positron emission tomography) scan ?

A

IT involves injecting a radioactive tracer (usually attached to glucose molecules) and taking images using a combination of a CT scanner and a gamma ray detector to visualise how metabolically active tissues are. They are useful in identifying areas that the cancer has spread to by showing areas of increased metabolically activity.

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9
Q

What is a bronchoscopy with EBUS ?

A

It involves endoscopy with ultrasound equipment on the end of the scope. This allows detailed assessment of the tumour and ultrasound guided biopsy.

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10
Q

How is a histological diagnosis of lung cancer obtained ?

A

A biopsy is performed either by bronchoscopy or percutaneously

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11
Q

What is the first line treatment for non-small cell lung cancer pts that have the disease isolated to a single area ? + what else can be curative ?

A

SURGERY

  • Lobectomy is first line
  • Segmentectomy or wedge resecting is also an option
  • Radiotherapy can also be curative
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12
Q

How can chemotherapy be used in relation to lung cancer (two ways) ?

A
  • It can be offered in addition to surgery or radiotherapy in certain pts to improve outcomes (“adjuvant chemotherapy”)
  • Or it can be used as palliative treatment to improve survival and quality of life in later stages of non-small cell lung cancer (“palliative chemotherapy”)
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13
Q

What is the most common treatment for small cell lung cancer ?

A

Usually chemotherapy and radiotherapy

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14
Q

Which type of lung cancer generally has the worse prognosis: NSCLC or SCLC ?

A

SCLC

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15
Q

What can be done as part of palliative treatment to relieve bronchial obstruction caused by lung cancer ?

A

Endobronchial treatment with stents or debulking

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16
Q

How does a recurrent laryngeal nerve palsy present + how can it be caused by a lung cancer ?

A
  • It presents with a hoarse voice

- It is caused by the cancer pressing on or affecting the recurrent laryngeal nerve as it passes through the mediastinum

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17
Q

How can a lung cancer cause a phrenic nerve palsy + how does this present ?

A
  • Due to nerve compression

- This causes diaphragm weakness and presents as shortness of breath.

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18
Q

How can a lung cancer cause superior vena cava obstruction, how does it present and what is Pemberton’s sign ?

A
  • It is caused by direct compression of the tumour on the SVC
  • It presents with facial swelling, difficulty breathing and distended veins in the neck and upper chest.
  • Pemberton’s sign is where raising the hands over the head causes facial congestion and cyanosis. This is a medical emergency.
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19
Q

What is Horner’s syndrome and how can it be caused by a lung cancer ?

A
  • It’s a triad of ptosis, anhidrosis and miosis

- It can be caused by a Pancoast tumour (tumour in the pulmonary apex) pressing on the sympathetic ganglion

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20
Q

Which type of lung cancer can cause SIADH and how does it present ?

A
  • Caused by ectopic ADH secretion by a small cell lung cancer
  • Presents with hyponatraemia (due to dilation of blood solutes)
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21
Q

Which type of lung cancer can cause Cushing’s syndrome/ how does it do this ?

A

Caused by ectopic ACTH secretion by a small cell lung cancer

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22
Q

Which type of lung cancer can cause hypercalcaemia and how does it do this ?

A

Caused by a ectopic parathyroid hormone from a squamous cell carcinoma

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23
Q

Which type of lung cancer can cause limbic encephalitis/ what is it, name four symptoms this could present with and what antibodies is it associated with ?

A

It’s a paraneoplastic syndrome where the SCLC causes the immune system to make antibodies to tissues in the brain, specifically the limbic system, causing inflammation in these areas. This causes symptoms such as short term memory impairment, hallucinations, confusion and seizures. It is associated with anti-Hu antibodies.

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24
Q

What is Lambert-Eaton myasthenic syndrome ?

A

Is a result of antibodies produced by the immune system against SCLC cells. These antibodies also target and damage voltage-gated calcium channels sited on the presynaptic terminals in motor neurones. This leads to weakness, particularly in the proximal muscles but can also affect intraocular muscles causing diplopia , levator muscles in the eyelid causing ptosis and pharyngeal muscles causing slurred speech and dysphagia. This weakness gets worse with prolonged use of the muscles.

This syndrome has similar symptoms to myasthenia gravis although the symptoms tend to be more insidious and less pronounced in Lambert-Eaton syndrome. In older smokers with symptoms of Lambert-Eaton syndrome consider a SCLC.

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25
Q

What is a mesothelioma, what’s is it linked to and what is the prognosis/treatment options ?

A

It is a lung malignancy affecting the mesothelial cells of the pleura. It is strongly linked to asbestos inhalation. There is a huge latent period between exposure to asbestos and the development of mesothelioma of up to 45 years. The prognosis is very poor. Chemotherapy can improve survival but it is essentially palliative.

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26
Q

What is pneumonia and how can it be seen ?

A

It is simply an infection of the lung tissue. It causes inflammation of the lung tissue and production of sputum that fills the airways and alveoli. Pneumonia can be seen as consolidation on a CXR.

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27
Q

What are the three different classifications of pneumonia ?

A
  • Community acquired pneumonia
  • Hospital acquired pneumonia (if it develops more than 48hrs after hospital admission)
  • Aspiration pneumonia
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28
Q

Presentation of pneumonia ? (seven points)

A
  • SOB
  • Cough productive of sputum
  • Fever
  • Haemoptysis (coughing up blood)
  • Pleuritic chest pain (sharp chest pain worse on inspiration)
  • Delirium
  • Sepsis
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29
Q

In pneumonia there may be a derangement in basic observations. These can indicate sepsis secondary to the pneumonia. Name 6 of these signs ?

A
  • Tachypnoea
  • Tachycardia
  • Hypoxia
  • Hypotension
  • Fever
  • Confusion
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30
Q

Characteristic chest signs when examining someone with pneumonia (three) ?

A
  • Bronchial breath sounds - these are harsh breath sounds equally loud on inspiration and expiration. These are caused by consolidation of the lung tissue around the airway.
  • Focal coarse crackles - these are caused by air passing through sputum in the airways similar to using a straw to blow air through a drink.
  • Dullness to percussion - due to lung tissue collapse and/ or consolidation
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31
Q

NICE recommend using the scoring system CRB-65 out of hospital and CURB 65 in hospital for assessing the severity of a pneumonia. What does CURB 65 stand for ?

A
C-Confusion
U-Urea >7
R-Respiratory rate > or equal to 30
B -BP < 90 systolic or < or equal to 60 diastolic
65 - Age > or equal to 65
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32
Q

What does the CURB 65 scoring mean ?

A

0/1 = Consider treatment at home
2 or above = Consider hospital admission
3 or above = Consider intensive care assessment

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33
Q

Common causes of pneumonia (two) ?

A
Streptococcus pneumoniae (50%)
Haemophilus influenzae (20%)
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34
Q

Other causes of pneumonia and associations (three) ?

A
  • Moraxella catarrhalis in immunocompromised pts or those with chronic pulmonary disease
  • Pseudomonas aeruginosa in pts with cystic fibrosis or bronchiectasis
  • Staphylococcus aureus in pts with cystic fibrosis
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35
Q

What is atypical pneumonia, what don’t they respond to and what can they be treated with ?

A

A pneumonia caused by an organism that cannot be cultured in the normal way or detected using a gram stain. They don’t respond to penicillins. They can be treated with macrolides, fluoroquines and tetracyclines.

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36
Q

Mnemonic for remembering 5 causes of atypical pneumonia + what does it stand for ?

A

Legions of psittaci MCQs

Legions - Legionella pneumophilia
Psittaci - Chlamydia psittaci
M - Mycoplasma pneumoniae
C - Chlamydophila penumoniae
Qs - Q fever (coxiella burnetii)
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37
Q

What is legionella pneumophila (Legionnaires’ disease) ?

A

An atypical pneumonia. Typically caused by infected water supplies or air conditioning units. It can cause hyponatraemia by causing an SIADH. The typical exam pt has recently had a cheap hotel holiday and presents with hyponatraemia.

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38
Q

What is mycoplasma pneumoniae ?

A

An atypical pneumoniae. This causes a milder pneumonia and can cause a rash called erythema multiforme characterised by varying sized “target lesions” formed by pink rings and pale centres. It can also cause neurological symptoms in young pts in the exams.

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39
Q

What is chlamydophilia pneumoniae ?

A

An atypical pneumonia. The presentation might be a school aged child with mild to moderate chronic pneumonia and wheeze. Be cautious though as this presentation is very common without chlamydophilia pneumoniae infection.

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40
Q

What is coxiella burnetii AKA “Q fever” ?

A

An atypical pneumonia. It is linked to exposure to animals and their bodily fluids. The MCQ pt is a farmer with a flu like illness.

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41
Q

What is chlamydia psittaci ?

A

An atypical pneumonia. This is typically contracted from contact with infected birds. The MCQ pt is a parrot owner.

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42
Q

What is pneumocystis jiroveci pneumonia (PCP), what pts does it occur in, how does it present and whats the treatment ?

A

It is a fungal pneumonia that occurs in pts that are immunocompromised. It is particularly important in pts with poorly controlled or new HIV with a low CD4 count. It usually presents subtly with a dry cough without sputum, SOB on exertion and night sweats. Treatment is with co-trimoxazole (trimethoprim/sulfamethoxazole), which is known by the brand name “Septrin”. Pts with low CD4 counts are prescribed prophylactic oral co-trimoxazole to protect against PCP.

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43
Q

Patients in the community with a CRB 0 or 1 pneumonia do not necessarily need investigations. NICE suggest considering a “point of care” test in primary care for CRP level to help guide management, however this is not widely available. If they arrive in hospital they will probably get a minimum of what (four) ?

A
  • CXR
  • FBC (raised white cells)
  • U&Es (urea)
  • CRP (raised in inflammation and infection)
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44
Q

Pts with moderate or severe cases of pneumonia should also have what (three) ?

A
  • Sputum cultures
  • Blood cultures
  • Legionella and pneumococcal urinary antigens (send a urine sample for antigen testing)
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45
Q

Inflammatory markers such as WBCs and CRP are roughly raised in proportion to the severity of the pneumonia infection. This trend can be helpful in monitoring progress of pts towards recovery. For example repeating WBC and CRP after 3 days of antibiotics may show a downward trend suggesting the antibiotics are working. Which of these two tests commonly shows a delayed response ?

A
  • CRP

- WBCs typically responds faster and gives a more “up to date picture”

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46
Q

What is the problem with using WBCs and CRP to look at how a pneumonia infection is progressing in an immunocompromised pt ?

A

Pts that are immunocompromised may not show an inflammatory response and may not have raised inflammatory markers despite severe infection.

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47
Q

When choosing antibiotics for pneumonia always follow your local area guidelines. These are developed by looking at the bacteria in your local area for their antibiotic resistance. Moderate or severe pneumonia or septic pts usually start with IV antibiotics. These are then changed to oral antibiotics guided by clinical improvement or improvement in their inflammatory markers. What are the typical antibiotic course lengths for mild CAP and moderate to severe CAP + what drugs might you give?

A

Mild CAP: 5 day course of oral antibiotics (amoxicillin or macrolide)
Moderate to severe CAP: 7-10 day course of dual antibiotics (amoxicillin and macrolide)

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48
Q

Name five complications of pneumonia ?

A
  • Sepsis
  • Pleural effusion
  • Lung abscess
  • Empyema
  • Death
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49
Q

What are lung function tests ?

A

They are used to help establish a diagnosis in lung disease. They are particularly helpful in obstructive and restrictive lung disease where there will be recognisable findings on the tests.

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50
Q

What is spirometry ?

A

A test used to establish objective measures of lung function. It involves different breathing exercises into a machine that measures volumes of air and flow rates and produces a report.

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51
Q

What is reversibility testing ?

A

It involves giving a bronchodilator to someone prior to repeating a spirometry test to see the impact this has on the results

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52
Q

What is FEV1 ?

A

Forced expiratory volume in 1 second. This is the volume of air a person can exhale as fast as they can in 1 second. This is a measure of how easily air can flow out of the lungs. It will be reduced if there is any air flow OBSTRUCTION.

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53
Q

What is FVC ?

A

Forced vital capacity. This is the total amount of air a person can exhale after a full inhalation. This is a measure of the total volume of air that the person can take in to their lungs. It will be reduced if there is any RESTRICTION on the capacity of their lungs.

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54
Q

How can obstructive lung disease be diagnosed ?

A

When the FEV1 is less than 75% of the FVC (FEV1:FVC ratio < 75%). This suggests that there is some obstruction slowing the passage of air getting out of the lungs. The person may have a relatively good lung volume but air is only able to move in and out of the lungs slowly due to obstruction.

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55
Q

In asthma what causes the obstruction ?

A

A narrowed airway due to bronchoconstriction.

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56
Q

In COPD what causes the obstruction

A

Chronic airway and lung damage.

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57
Q

Are asthma and COPD reversible (i.e. by giving a bronchodilatory)?

A

Asthma is typically reversible but COPD isn’t

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58
Q

If FEV1 and FVC are equally reduced and the FEV1:FVC ratio > 75% what does this suggest ?

A

Restrictive lung disease

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59
Q

What is restrictive lung disease ?

A

Where there is a restriction in the ability of the lungs to expand and fill with air. The lungs are restricted from effectively expanding. This can be differentiated from obstructive lung disease where there is obstruction of air flow through the airways in to and out of the lungs. This restriction of lung expansion leads to inadequate ventilation of the alveoli and therefore inadequate oxygenation of the blood.

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60
Q

Why will restrictive lung disease cause the FEV1/FVC ratio to be normal or raised ?

A

As there is no obstructive pathology present that would be affecting air flow through the airways. The FVC will be reduced because there is a restriction of the overall expansion and thus maximum capacity of the lungs.

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61
Q

5 causes of restrictive lung disease ?

A
  • Interstitial lung disease e.g. pulmonary fibrosis
  • Sarcoidosis
  • Obesity
  • MND
  • Scoliosis
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62
Q

What is peak flow, how is it measured and what disease is it useful in looking at ?

A

It is a measurement of the fastest point of a persons expiratory flow of air. It is a simple way of demonstrating how much obstruction to airflow is present in a pts lungs. It is measured using a peak flow meter. It is useful in obstructive lung disease, particularly asthma, to measure how well the asthma is controlled and how severe an acute exacerbation is.

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63
Q

Technique for peak flow ?

A

To stand tall, take a deep breath in, make a good seal around the device with the lips and blow as fast and hard as possible into the device. Take three attempts and record the best result.

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64
Q

How is peak flow recorded and why ?

A

It is usually recorded as a % of predicted. The predicted peak flow can be obtained based on sex, height and age using a reference chart. Peak flow varies dramatically based on the size and age of the pt.

For example an asthmatic pt with a predicted peak flow of 400 that only manages a score of 200 on their best attempt of 3 currently has a peak flow at 50% of predicted.

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65
Q

What is asthma ?

A

A chronic inflammatory condition of the airways that causes episodic exacerbations of bronchoconstriction. This narrowing of the airways causes an obstruction to airflow going in and out of the lungs. This airway obstruction is reversible and typically responds to bronchodilators such as salbutamol. The bronchoconstriction is caused by airway hypersensitivity and can be triggered by environmental factors.

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66
Q

Name 6 typical triggers of asthma ?

A
  • Infection
  • Night time or early morning
  • Exercise
  • Animals
  • Cold, damp or dusty air
  • Strong emotions
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67
Q

Presentation suggesting a diagnosis of asthma (6 bullet points)?

A
  • Episodic symptoms
  • Diurnal variability. Typically worse at night
  • Dry cough with wheeze and SOB
  • A history of other atopic conditions such as eczema, hayfever and food allergies.
  • Family history
  • Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
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68
Q

Presentation indicating a diagnosis other than asthma (5 bullet points) ?

A
  • Wheeze related to coughs and colds more suggestive of viral induced wheeze
  • Isolated or productive cough
  • Normal investigations
  • No response to treatment
  • Unilateral wheeze. This suggests a focal lesion or infection.
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69
Q

BTS/Sign guidelines (2016) on diagnosis of asthma ?

A
  • High probability of asthma clinically: Try treatment
  • Intermediate probability of asthma: Perform spirometry with reversibility testing
  • Low probability of asthma: Consider referral and investigating for other causes.
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70
Q

NICE guidelines (2017) on diagnosis of asthma ?

A

NICE recommend assessment and testing at a “diagnostic hub” to establish a diagnosis. They specifically advise not to make a diagnosis clinically and require investigations.

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71
Q

First line investigations for asthma (two) ?

A
  • Fractional exhaled nitric oxide

- Spirometry with bronchodilator reversibility

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72
Q

In asthma if there is diagnostic uncertainty after first line investigations these can be followed up with what further testing (two) ?

A
  • Peak flow variability measured by keeping a diary of peak flow measurements several times per day for 2 to 4 weeks.
  • Direct bronchial challenge test with histamine or methacholine.
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73
Q

How do Short acting beta 2 adrenergic receptor agonists work ?

A

They act cause smooth muscles of the airways the relax resulting in bronchodilation

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74
Q

How do long-acting beta 2 agonists (LABA) work ?

A

Same as SABAs but have a much longer action

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75
Q

Why are ICSs e.g. betclometasone effective in treating asthma ?

A

They reduce the inflammation and reactivity of the airways

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76
Q

How do long-acting muscarinic antagonists (LAMA) e.g. tiotropium work ?

A

They block the acetylcholine receptors. Acetylcholine receptors are stimulated by the parasympathetic nervous system and cause contraction of the bronchial smooth muscles therefore blocking these receptors leads to bronchodilation

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77
Q

How do leukotriene receptor antagonists e.g. montelukast work ?

A

Leukotrienes are produced by the immune system and cause inflammation, bronchoconstriction and mucus secretion in the airways. Leukotriene receptor antagonists work by blocking the effects of leukotrienes..

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78
Q

How does theophylline work ?

A

By relaxing bronchial smooth muscle and reducing inflammation. Unfortunately it has a narrow therapeutic window and can be toxic in excess so monitoring plasma theophylline levels is required. This is done 5 days after starting treatment and 3 days after each dose change

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79
Q

What is maintenance and reliever therapy (MART) ?

A

This is a combination inhaler containing a low dose inhaled corticosteroid and a fast acting LABA. This replaces all other inhalers and the pt uses this single inhaler both regularly as a “preventer” and also as a “reliever” when they have symptoms.

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80
Q

Principles of using asthma stepwise ladder (five) ?

A
  • Start at the most appropriate step for the severity of the symptoms
  • Review at regular intervals based on severity
  • Step up and down the ladder based on symptoms
  • Aim to achieve no symptoms or exacerbations on the lowest dose and number of treatments (This is often difficult in practice)
  • Always check inhaler technique and adherence at review
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81
Q

NICE guidelines stepwise ladder for asthma treatment (8 steps) ?

A
  1. Add a SABA inhaler e.g. salbutamol as required for infrequent wheezy episodes.
  2. Add a regular low dose inhaled corticosteroid
  3. Add an oral leukotriene receptor antagonist i.e. montelukast
  4. Add LABA inhaler e.g. salmeterol. Continue the LABA only if the pt has a good response.
  5. Consider changing to a maintenance and reliever therapy (MART) regime
  6. Increase the inhaled corticosteroid to a moderate dose
  7. Consider increasing the inhaled corticosteroid dose to a high dose or oral theophylline or an inhaled LAMA e.g. imotropium
  8. Refer to a specialist
82
Q

Additional management of asthma (4 points) ?

A
  • Each pt should have an individual asthma self-management programme
  • Yearly flu jab
  • Yearly asthma review
  • Advise exercise and avoid smoking
83
Q

Acute exacerbation of asthma ?

A

Rapid deterioration in symptoms

84
Q

Presentation of an acute exacerbation of asthma (5 bullet points) ?

A
  • Progressively worsening SOB
  • Use of accessory muscles
  • Tachypnoea
  • Symmetrical expiratory wheeze on auscultation
  • The chest can sound “tight” on auscultation with reduced air entry
85
Q

Moderate acute asthma ?

A

PEFR 50 - 75% predicted

86
Q

Severe acute asthma ?

A
  • PEFR 33 - 50% predicted
  • RR > 25
  • HR > 110
  • Unable to complete sentences
87
Q

Life-threatening acute asthma (five bullet points) ?

A
  • PEFR < 33%
  • Sats < 92%
  • Becoming tired
  • No wheeze. This occurs when the airways are so tight there is no air entry at all. This is ominously described as a “silent chest”
  • Haemodynamic instability (i.e. shock)
88
Q

Treatment for moderate acute asthma (four points) ?

A
  • Nebulised beta-2 agonists (i.e. salbutamol 5mg repeated as often as required)
  • Nebulised ipratropium bromide
  • STEROIDS. Oral prednisolone or IV hydrocortisone. These are usually continued for 5 days.
  • Antibiotics if there is convincing evidence of bacterial infection.
89
Q

Treatment for severe acute asthma (three points) ?

A
  • Oxygen if required to maintain sats 94-98%
  • Aminophylline infusion
  • Consider IV salbutamol
90
Q

Treatment for life threatening asthma (three points) ?

A
  • IV mangesium sulphate infusion
  • Admission to HDU or ICU
  • Intubation in the worst cases. This decision should be made early because it is very difficult to intubate with severe bronchoconstriction
91
Q

Will acute asthma pts initially have a respiratory alkalosis or respiratory acidosis and why ?

A

Respiratory alkalosis as tachypnoea causes a drop in CO2.

92
Q

Why is a normal pCO2 or hypoxia a concerning sign in acute asthma ?

A

It means the pt is tiring and indicates life threatening asthma. A respiratory acidosis due to high CO2 is also a very bad sign.

93
Q

How do you monitor a pts response to treatment of acute asthma (five points) ?

A
  • RR
  • Respiratory effort
  • Peak flow
  • Oxygen saturations
  • Chest auscultation
94
Q

Why do you need to monitor serum potassium when a pt is on salbutamol + what else does it cause ?

A

Salbutamol causes potassium to be absorbed from the blood into the cells. It also causes tachycardia.

95
Q

Plan after asthma attack ?

A

Optimise asthma control after an attack. Discharge pts with an “asthma action plan” that provides them with a clear plan for everything they need to know about their asthma in one place. Consider prescribing a “rescue pack” of steroids for the person to initiate in the future if they have another exacerbation of asthma. NICE suggest referral to a respiratory specialist after 2 attacks in 12 months.

96
Q

What is COPD ?

A

A non-reversible, long term deterioration in air flow through the lungs caused by damage to lung tissue. This lung damage is almost always the result of smoking. The damage to the lung tissues causes an obstruction to the flow of air through the airways making it more difficult to ventilate the lungs and making them more prone to developing infections.

97
Q

COPD exacerbations are often triggered by infections, what are these called ?

A

Infective exacerbations

98
Q

Presentation of COPD ?

A

Suspect COPD in a long term smoker presenting with chronic SOB, cough, sputum production, wheeze and recurrent respiratory infections, particularly in winter.

Always consider differential diagnoses such as lung cancer, fibrosis or heart failure. COPD does not cause clubbing. It is unusual for it to cause haemoptysis or chest pain. These symptoms should be investigated for a different cause.

99
Q

MRC dyspnoea scale ?

A

Grade 1 - Breathless on strenuous exercise
Grade 2 - Breathless on walking up hill
Grade 3 - Breathlessness that slows walking on the flat
Grade 4 - Stop to catch their breath after walking 100 metres on the flat
Grade 5 - Unable to leave the house due to breathlessness

100
Q

Diagnosis of COPD ?

A

Based on clinical presentation plus spirometry

Spirometry will show an “obstructive picture”. This means the overall lung capacity is not as bad as the pts ability to quickly blow air out of their lungs which is limited due to the damage to their airways causing airway obstruction.

Therefore in COPD:
FEV1/FVC ratio < 0.7

The obstructive picture does not show a dramatic response to reversibility testing with beta-2 agonist such as salbutamol during spirometry testing. If there is a large response to reversibility testing then consider asthma as an alternative diagnosis.

101
Q

How can severity of airflow obstruction be graded using the FEV1 ?

A

Stage 1: FEV1 >80% of predicted
Stage 2: FEV1 50-79% of predicted
Stage 3: FEV1 30-49% of predicted
Stage 4: FEV1 <30% of predicted

102
Q

Other investigations to consider to help with COPD diagnosis and management and to exclude other conditions (8 bullet points) ?

A
  • CXR - to exclude other pathology such as lung cancer
  • FBC - for polycythaemia or anaemia. Polycythaemia is a response to chronic hypoxia.
  • BMI - as a baseline to later assess weight loss (e.g. a cancer or severe COPD) or weight gain (e.g. steroids)
  • Sputum culture - to assess for chronic infections such as pseudomonas
  • ECG and echocardiogram to assess heart function
  • CT thorax for alternative diagnosis such as fibrosis, cancer or bronchiectasis
  • Serum alpha-1 antitrypsin - to look for alpha-1 antitrypsin deficiency. Deficiency leads to early onset and more severe disease.
  • Transfer factor for carbon monoxide (TLCO) - is decreased in COPD. It can give an indication about the severity of the disease and may be increased in other conditions such as asthma.
103
Q

Long term management of COPD

A

It is essential for pts to stop smoking. Pts should also have the pneumococcal and annual flu vaccine.

Step 1:
Short acting bronchodilators: beta-2 agonists (salbutamol or terbutaline) or short acting antimuscarinics (ipratropium bromide).

Step 2:
If they do NOT have asthmatic or steroid responsive features they should have a combined LABA plus a LAMA . “Anoro ellipta”, “Ultibro Breezhaler” and “DuaKlir Genuair” are examples of combination inhalers.

If they have asthmatic or steroid responsive features they should have a combined LABA and ICS. “Fostair”, “Symbicort” and “Seretide” are examples of combination inhalers. If these don’t work they can step up to a combination of LABA, LAMA and ICS. “Trimbo” and “Trelegy Ellipta” are xamples of LABA, LAMA and ICS combination inhalers.

104
Q

Additional management options in more severe cases of COPD (five bullet points) ?

A
  • Nebulisers (salbutamol and/or ipratroprium)
  • Oral theopylline
  • Oral mucolytic therapy to break down sputum (e.g. carbocisteine)
  • Long term prophylactic antibiotics (e.g. azithromycin)
  • Long term oxygen therapy at home - Used when severe COPD is causing problems such as chronic hypoxia, polycythaemia, cyanosis or cor pulmonale. It can’t be used if they smoke.
105
Q

What normally causes an exacerbation of COPD ?

A

Viral or bacterial infection

106
Q

Investigations for exacerbation of COPD (6)?

A
  • CXR - to look for pneumonia or other pathology
  • ECG - to look for arrhythmias or evidence of heart strain
  • FBC - to look for infection (raised white cells)
  • U&Es - to check electrolytes, which can be affected by infections and medications
  • Sputum culture - if significant infection is present
  • Blood cultures - if septic
107
Q

Why do you need to be particularly careful when giving oxygen to someone with COPD and how is this guided ?

A

Too much oxygen in someone that is prone to retaining CO2 can depress their respiratory drive. This slows their breathing rate and effort and leads to them retaining more CO2. Therefore, in someone who retains CO2 the amount of oxygen that is given needs to be carefully balanced to optimise their pO2 whilst not increasing their pCO2. This is guided by oxygen saturations and repeat ABGs.

108
Q

General rule (two rules) regarding target oxygen saturations in COPD ?

A
  • If retaining CO2 aim for oxygen saturations of 88-92% titrated by a venturi mask
  • If not retaining CO2 and their bicarbonate is normal (meaning they do not normally retain CO2) then give oxygen to aim for oxygen saturations above 94%
109
Q

Medical treatment of a COPD exacerbation ?

A

If well enough to remain at home:

  • Prednisolone 30mg once daily for 7-14 days
  • Regular inhalers or home nebulisers
  • Antibiotics if there is evidence of infection

In hospital:

  • Nebulised bronchodilators (e.g. salbumtamol 5mg/4h and ipratropium 500mcg/6h)
  • Steroids (e.g. 200mg hydrocortisone or 30-40mg oral prednisolone)
  • Antibiotics if evidence of infection
  • Physiotherapy can help clear sputum
110
Q

Treatment options in severe cases of exacerbated COPD not responding to first line treatment (4 bullet points) ?

A
  • IV aminophylline
  • Non-invasive ventilation (NIV)
  • Intubation and ventilation with admission to intensive care
  • Doxapram can be used as a respiratory stimulant where NIV or intubation is not appropriate
111
Q

What is non-invasive ventilation ?

A

It is used to support the lungs in respiratory failure secondary to obstructive lung disease. It is an alternative to full intubation and ventilation. Intubation and ventilation involves giving the pt a general anaesthetic, putting a plastic tube into the trachea and ventilating the lungs artificially. NIV involves using a full face mask or a tight fitting nasal mask to blow air forcefully into the lungs and ventilate them without having to intubate the pt. It is not pleasant, however it is much less invasive than intubation and ventilation and acts as a useful middle point between basic oxygen and intubation.

112
Q

What are the two types of NIV ?

A

BiPAP or CPAP

113
Q

What does BiPAP stand for ?

A

Bilevel postive airway pressure

114
Q

What does BiPAP involve ?

A

A cycle of high and low pressure to correspond to the pts inspiration and expiration

115
Q

When is BiPAP used + criteria for initiating it ?

A

It is used when there is type 2 respiratory failure, typically due to COPD.

The criteria for initiating BiPAP are:
-Respiratory acidosis (pH < 7.35, PaCO2 > 6) DESPITE adequate medical treatment

116
Q

Who would make the decision to initiate BiPAP ?

A

A registrar or above

117
Q

Main contraindications to BiPAP and what should be done to rule this out?

A

An untreated pneumothorax or any structural abnormality or pathology affecting the face, airway or GI tract. Pts should have a CXR prior to NIV to exclude pneumothorax where this does not cause a delay.

118
Q

What should happen if the NIV fails ?

A

There should be a plan in place so that everyone agrees whether the pt should process to intubation and ventilation and ICU or whether palliative care is more appropriate.

119
Q

IPAP and EPAP ?

A

Inspiratory positive airway pressure (IPAP) - is the pressure during inspiration. This is where air is forced into the lungs.

Expiratory positive airway pressure (EPAP) - is the pressure during expiration. This provides some pressure during expiration so that the airways don’t collapse. It helps air to escape the lungs in pts with obstructive lung disease.

120
Q

When should you check ABGs regarding BiPAP ?

A

1 hour after every change in pressure and 4 hours after that until stable. The IPAP is increased by 2-5cm increments until the acidosis resolves (pressures are measured in cm of water)

121
Q

What does CPAP stand for, what is it and why is it used ?

A

Continuous positive airway pressure. It provides continuous air blown into the lungs that keeps the airways expanded so that air can more easily travel in and out. It is used to maintain the pts airway in conditions where it is prone to collapse.

122
Q

Indications for CPAP (three) ?

A
  • Obstructive sleep apnoea
  • Congestive cardiac failure
  • Acute pulmonary oedema.
123
Q

What is interstitial lung disease ?

A

An umbrella term to describe conditions that affect the lung parenchyma (portion of the lung involved in gas transfer) causing inflammation and fibrosis. Fibrosis involves the replacement of the normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively.

124
Q

Diagnosis of ILD ?

A

Requires a combination of clinical features and a high resolution CT scan of the thorax. HRCT shows a “ground glass” appearance with ILD. When a diagnosis is unclear lung biopsy can be used to take samples of lung tissue and confirm the diagnosis on histology

125
Q

Prognosis + management of ILD ?

A

Generally there is a poor prognosis and limited management options in ILD as the damage is irreversible. Generally the treatment is supportive. Options are:

  • Remove or treat the underlying cause
  • Home oxygen where they are hypoxic at rest
  • Stop smoking
  • Physiotherapy and pulmonary rehabilitation
  • Pneumococcal and flu vaccine
  • Advanced care planning and palliative care where appropriate
  • Lung transplant is an option but the risks and benefits need careful consideration
126
Q

What is idiopathic pulmonary fibrosis , how does it present, who does it usually affect, what can examination show and what is the prognosis like ?

A

A type of ILD. There is progressive pulmonary fibrosis with no clear cause. It presents with an insidious onset of SOB and dry cough over more than 3 months. It usually affects adults over 50 years old. Examination can show bibasal fine inspiratory crackles and finger clubbing. Prognosis is poor with a life expectancy of 2-5 years from diagnosis.

127
Q

Two medications that are licensed for idiopathic pulmonary fibrosis ?

A
  • Pirfenidone - its an antifibrotic and anti-inflammatory

- Nintedanib - its a monoclonal antibody targeting tyrosine kinase

128
Q

Name four drugs that can cause drug induced pulmonary fibrosis ?

A
  • Amiodarone
  • Cyclophosphamide
  • Methotrexate
  • Nitrofurantoin
129
Q

Four causes of secondary pulmonary fibrosis ?

A
  • Alpha-1 antitripsin deficiency
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Systemic sclerosis
130
Q

What is hypersensitivity pneumonitis (AKA extrinsic allergic alveolitis ) ?

A

A type of ILD. It’s a type III hypersensitivity reaction to an environmental allergen. It causes parenchymal inflammation and destruction in people that are sensitive to that allergen.

131
Q

How would you investigate for hypersensitivity pneumonitis ?

A

By performing a bronchoalveolar lavage which involves collecting cells from the airway during bronchoscopy by washing the airways with fluid then collecting that fluid for testing. This shows raised lymphocytes and mast cells in hypersensitivity pneumonitis.

132
Q

Management of hypersensitivity pneumonitis ?

A

By removing the allergen, giving oxygen where necessary and steroids.

133
Q

Four examples of hypersensitivity pneumonitis ?

A

Bird-fanciers lung - is a reaction to bird droppings
Farmers lung - is a reaction to mouldy spores in hay
Mushroom workers’ lung - is reaction to specific mushroom antigens
Malt workers lung - is a reaction to mould on barley

134
Q

What is cryptogenic organising pneumonia ?

A

A type of ILD. It involves a focal area of inflammation of the lung tissue. This can be idiopathic or triggered by infection, inflammatory disorders, medications, radiation or environmental toxins or allergens.

135
Q

Presentation of cryptogenic organising pneumonia ?

A

Very similar to infectious pneumonia with SOB, cough, fever and lethargy. It also presents similarly to pneumonia on a CXR with focal consolidation

136
Q

Diagnosis + treatment of cryptogenic organising pneumonia ?

A

Diagnosis is often delayed due to similarities to infective pneumonia. Lung biopsy is the definitive investigation.

Treatment is with systemic corticosteroids.

137
Q

What is asbestosis ?

A

Its a type of ILD. It’s lung fibrosis related to the inhalation of asbestos. Asbestos is fibrogenic meaning it causes lung fibrosis. It is also oncogenic. The effects of asbestos usually take several decades to develop.

138
Q

Name four problems asbestos inhalation causes ?

A
  • Lung fibrosis
  • Pleural thickening and pleural plaques
  • Adenocarcinoma
  • Mesothelioma
139
Q

What is a pleural effusion ?

A

A collection of fluid in the pleural cavity.

140
Q

What defines an exudative vs transudative pleural effusion ?

A
Exudative = high protein count (more than 3g/L)
Transudative = relatively lower protein count (less than 3g/L).

Whether it is exudative or transudative helps determine the cause.

141
Q

What are exudative causes of a pleural effusion related to and name four of these causes ?

A

They are related to inflammation. The inflammation results in protein leaking out of the tissues into the pleural space (ex-meaning moving out of). Think of the causes of inflammation:

  • Lung cancer
  • Pneumonia
  • Rheumatoid arthritis
  • Tuberculosis
142
Q

What are transudative causes of a pleural effusion related to and name four of these causes ?

A

They relate to fluid moving across into the pleural space (trans-meaning moving across). Think of the causes of fluid shifting:

  • Congestive cardiac failure
  • Hypoalbuminaemia
  • Hypothyroidism
  • Meig’s syndrome (right sided pleural effusion with ovarian malignancy)
143
Q

Presentation of a pleural effusion (four signs + symptoms) ?

A
  • SOB
  • Dullness to percussion over the effusion
  • Reduced breath sounds
  • Tracheal deviation away from the effusion if it is massive
144
Q

Investigations for pleural effusion ?

A

CXR shows:

  • Blunting of the costophrenic angle
  • Fluid in the lung fissures
  • Larger effusions will have a meniscus. This is a curving upwards where it meets the chest wall and mediastinum.
  • Tracheal and mediastinal deviation if its is a massive effusion

Taking a sample of the pleural fluid by aspiration or chest drain is required to analyse it for protein count, cell count, pH, glucose, LDH and microbiology testing

145
Q

Treatment of a pleural effusion ?

A

Conservative management may be appropriate as small effusions will resolve with treatment of the underlying cause. Larger effusions often need aspirations or drainage.

Pleural aspiration involves sticking a needle through the chest wall into the effusion and aspirating the fluid. this can temporarily relieve the pressure but the effusion may recur and repeated aspiration may be required.

Chest drain can be used to drain the effusion and prevent it recurring

146
Q

What is empyema, which pts should you suspect it in, what would a pleural aspiration show and how do you treat it ?

A

It’s where there is an infected pleural effusion.

Suspect empyema in a pt who has an improving pneumonia but new or ongoing fever.

Pleural aspiration shows pus, acidic pH (pH < 7.2), low glucose and high LDH.

Empyema is treated by chest drain to remove the ps and antibiotics

147
Q

What is a pneumothorax ?

A

When air gets into the pleural space separating the lung from the chest wall.

148
Q

How does the typical pneumothorax pt in exams present ?

A

A tall, young man presenting with sudden breathlessness and pleuritic chest pain (sudden sharp and stabbing pain).

149
Q

Name four causes of a pneumothorax ?

A
  • Spontaneous
  • Trauma
  • Iatrogenic, for example due to lung biopsy, mechanical ventilation or central line insertion
  • Lung pathology such as infection, asthma or COPD
150
Q

Investigations for pneumothorax ?

A
  • Erect CXR - is the investigation of choice for a simple pneumothorax. It shows an area between the lung tissue and the chest wall where there are no lung markings. There will be a line demarcating the edge of the lung where the lung markings ends and the pneumothorax begins. Measuring the size of the pneumothorax on a CXR can be done according to the BTS guidelines from 2010. This involves measuring horizontally from the lung edge to the inside of the chest wall at the level of the hilum.
  • CT thorax - can detect a pneumothorax that is too small on a CXR. It can also be used to accurately assess the size of the pneumothorax.
151
Q

Management of a pneumothorax (four bullet points) ?

A
  • If no SOB and there is a < 2cm rim of air on the CXR then no treatment is required as it will spontaneously resolve. Follow up in 2 - 4 weeks is recommended.
  • If SOB and/or there is > 2cm rim of air on the CXR then it will require aspiration and reassessment.
  • If aspiration fails twice it will require a chest drain.
  • Unstable pts or bilateral or secondary pneumothoraces generally require a chest drain
152
Q

What is a tension pneumothorax ?

A

A pneumothorax caused by trauma to the chest wall which creates a one way valve that lets air in but not out of the pleural space. the one way valve means that during inspiration air is drawn in to the pleural space and during expiration air is trapped in the pleural space. Therefore, more air keeps getting drawn in to the pleural space with each breath and cannot escape. This is dangerous as it creates pressure inside the thorax that will push the mediastinum across, kink the big vessels in the mediastinum and cause cardiorespiratory arrest.

153
Q

5 signs of a tension pneumothorax ?

A
  • Tracheal deviation away form side of the pneumothorax
  • Reduced air entry on the affected side
  • Increased resonant to percussion on the affected side
  • Tachycardia
  • Hypotension
154
Q

Management of a tension pneumothorax ?

A

“Insert a large bore cannula into the second IC space in the mid clavicular line”

If a tension pneumothorax is suspected do not wait for any investigations. Once the pressure is relieved with a cannula then a chest drain is required for definitive management.

Chest drains are inserted into the “triangle of safety”. This triangle is formed by:

  • The 5th ICS (or inferior nipple line)
  • The mid axillary line (or lateral edge of latissimus dorsi)
  • The anterior axillary line (or the lateral edge of pectoris major)

The needle is inserted just above the rib to avoid the neurovascular bundle that runs just below the rib. Once the chest drain is inserted, obtain a CXR to check the positioning.

155
Q

What is a pulmonary embolism (PE) and what normally causes it ?

A

A condition where a thrombus forms in the pulmonary arteries. This is usually the result of a DVT that developed in the legs and embolised through the venous system and the right side of the heart to the pulmonary arteries. Once in the pulmonary arteries the thrombus will block blow to the lung tissue and create strain on the right side of the heart. DVTs and PEs are collectively known as venous thromboembolism (VTE)

156
Q

Risk factors for developing a DVT or PE (9 bullet points) ?

A
  • Immobility
  • Recent surgery
  • Long haul flights
  • Pregnancy
  • Hormone therapy with oestrogen
  • Malignancy
  • Polycythaemia
  • Systemic lupus erythematosus
  • Thrombophilia
157
Q

Every pt admitted to hospital should be assessed for their risk of VTE. If they are at increased risk of VTE they should receive prophylaxis with what unless contraindicated ?

A

Low molecular weight heparin e.g. enoxaparin

158
Q

Name 2 contraindications to giving LMWH as prophylaxis to prevent a VTE ?

A

Active bleeding or existing anticoagulation with warfarin or a NOAC

159
Q

What else can be used to prevent a VTE in an at risk pt besides LMWH and what would contraindicate this ?

A
  • Anti embolic compression stockings

- Main contraindication is significant peripheral arterial disease

160
Q

Presentation of Pulmonary Embolism ?

A

Can be subtle. In pts with potential features of a PE, risk factors for PE and no other explanation for their symptoms, have a low threshold for suspecting a PE. Presenting features include:

  • SOB
  • Cough with or without haemoptysis
  • Pleuritic chest pain
  • Hypoxia
  • Tachycardia
  • Raised RR
  • Low grade fever
  • Haemodynamic instability causing hypotension

There may also be S+S of a DVT such as unilateral leg swelling and tenderness.

161
Q

What is the wells score ?

A

It predicts the risk of a pt presenting with symptoms actually having a DVT or pulmonary embolism. It takes in to account risk factors such as recent surgery and clinical findings such as tachycardia (HR > 100) and haemoptysis.

162
Q

Diagnosis of PE ?

A

NICE recommend assessing for alternative causes with a:

  • History
  • Examination
  • CXR

Perform a Wells score and proceed based on the outcome:

  • LIKELY: perform a CT pulmonary angiogram
  • UNLIKELY: perform a d-dimer and if positive perform a CTPA
163
Q

What is a d-dimer test + what other conditions can cause a raised d-dimer?

A

A d-dimer is a fibrin degradation product. A d-dimer test is a sensitive (95%) but not specific blood test for VTE. This makes it useful for excluding VTE where there is a low suspicion. It is almost always raised if there is a DVT, however other conditions can also cause a raised d-dimer:

  • Pneumonia
  • Malignancy
  • Heart failure
  • Surgery
  • Pregnancy
164
Q

There are two main options for establishing a definitive diagnosis of a PE: CT pulmonary angiogram or ventilation-perfusion (VQ) scan.

What is a CTPA and why is it usually first choice for investigation a PE?

A

It involves a chest CT scan with an intravenous contrast that highlights the pulmonary arteries to demonstrate any blood clots. This is usually the first choice for investigating a PE as it tends to be more readily available, provides a more definitive assessment and gives information about alternative diagnoses such as pneumonia or malignancy.

165
Q

There are two main options for establishing a definitive diagnosis of a PE: CT pulmonary angiogram or ventilation-perfusion (VQ) scan.

What is a VQ scan (include which pts they are used in) ?

A

It involves using radioactive isotopes and a gamma camera to compare the ventilation with the perfusion of the lungs. They are used in pts with renal impairment, iodinated contrast allergy or at risk from radiation, where a CTPA is unsuitable (CTPAs expose pts to a higher level of radiation). First, the isotopes are inhaled to fill the lungs and a picture is taken to demonstrate ventilation. Next a contrast containing isotopes is injected and a picture is taken to demonstrate perfusion. The two pictures are compared. With a PE there will be a deficit in perfusion as the thrombus blocks blood flow to the lung tissue. This area of lung tissue will be ventilated but not perfused.

166
Q

Pts with a PE often have a respiratory alkalosis due to the high RR. The other main cause is hyperventilation syndrome. How would you differentiate between the two from an ABG ?

A

Pts with a PE will have a low pO2 whereas pts with hyperventilation syndrome will have a high pO2.

167
Q

Supportive management of a PE (four bullet points) ?

A
  • Admission to hospital
  • Oxygen as required
  • Analgesia as required
  • Adequate monitoring for any deterioration
168
Q

Initial management of a PE ?

A

LMWH. This should be started immediately before confirming the diagnosis in pts where DVT or PE is suspected and there is a delay in getting the scan. Examples are enoxaparin and dalteparin.

169
Q

What are the options for long term anticoagulation in VTE ?

A

Warfarin, a NOAC or LMWH

When switching to warfarin continue LMWH for 5 days or until the INR is between 2 and 3 (target INR) for 24hrs on warfarin (whichever is longer)

170
Q

What is the first line long term anticoagulation treatment following a VTE in pregnancy or cancer ?

A

LMWH

171
Q

How long should you continue anticoagulation for following a VTE (3 bullet points) ?

A
  • 3 months - if there is an obvious reversible cause (then review)
  • Beyond 3 months - if the cause is unclear, there is recurrent VTE or there is an irreversible underlying cause such as thrombophilia. This is often 6 months in practice.
  • 6 months in active cancer (then review)
172
Q

When would thrombolysis be performed in regards to a PE ?

A

When the PE is massive with haemodynamic compromise. There is a significant risk of bleeding which can make it dangerous so basically its only used where the benefits outweigh risks like the situation mentioned at the start.

173
Q

Two way thrombolysis can be performed to treat a massive PE ?

A
  • Intravenously using a peripheral cannula

- Directly into the pulmonary arteries using a central catheter. This is called catheter-directed thrombolysis .

174
Q

What is catheter-directed thrombolysis?

A

A catheter is inserted into the venous system, through the right side of the heart and into the pulmonary arteries. The operator can then administer the thrombolytic agent directly into the location of the thrombus. Special equiptment can also be used to physically break down the thrombus and aspirate it. There is a risk of damaging the pulmonary arteries doing this

175
Q

What is pulmonary hypertension ?

A

Increased resistance and pressure on blood in the pulmonary arteries. This causes a strain on the right side of the heart trying to pump blood through the lungs. this also causes a back pressure of blood into the systemic venous system.

176
Q

Grouped causes of pulmonary hypertension with examples ?

A

Group 1 - Primary pulmonary hypertension or connective tissue disease such as systemic lupus erythematous (SLE)
Group 2 - Left heart failure usually due to MI or systemic hypertension
Group 3 - Chronic lung disease such as COPD
Group 4 Pulmonary vascular disease such as pulmonary embolism
Group 5 - Miscellaneous causes such as sarcoidosis, glycogen storage disease and haematological disorders.

177
Q

S+S of pulmonary hypertension ?

A

SOB is main presenting symptom. Other S+S are:

  • Syncope
  • Tachycardia
  • Raised JVP
  • Hepatomegaly
  • Peripheral oedema
178
Q

Investigations for pulmonary hypertension ?

A

ECG changes

  • RV hypertrophy seen as large R waves on the right sided chest leads (V1-3) and S waves on the left sided chest leads (V4-6)
  • Right axis deviation
  • Right BBB (MaRRoW)

CXR changes:

  • Dilated pulmonary arteries
  • RV hypertrophy

Other investigations

  • A raised NT-proBNF blood test result indicates right ventricular failure
  • Echo can be used to estimate pulmonary artery pressure
179
Q

Prognosis + management of PH ? (include management for PPH, SPH and supportive treatment)

A

Prognosis is quite poor with a 30-40% 5 year survival from diagnosis. This can increase to 60-70% where specific treatment is possible.

PPH can be treated with:

  • IV prostanoids (e.g. epoprostenol)
  • Endothelin receptor antagonists (e.g. macitentan)
  • Phosphodiesterase-5 inhibitors (e.g. sildenafil)

SPH is managed by treating the underlying cause, such as PE or SLE

Supportive treatment for complications such as resp failure, arrhythmias and HF.

180
Q

What is sarcoidosis ?

A

A granulomatous inflammatory condition

181
Q

What are granulomas ?

A

Nodules of inflammation full of macrophages

182
Q

What is sarcoidosis associated with ?

A

It is usually associated with chest symptoms but also has multiple extra-pulmonary manifestations such as erythema nodosum and lymphadenopathy.

183
Q

How often does sarcoidosis cause symptoms ?

A

Symptoms can vary dramatically from asymptomatic (in up to 50%) to severe and life threatening.

184
Q

Incidence of sarcoidosis ?

A

There are two spikes in incidence, in young adulthood and again around age 60. Women are affected more often and it occurs more frequently in black people compared with other ethnic groups

185
Q

Typical sarcoidosis MCQ exam pt ?

A

20-40 yr old black female presenting with a dry cough and SOB. They may have nodules on their shins suggesting erythema nodosum.

186
Q

Organs affected by sarcoidosis ?

A

Lungs (affecting over 90%)

  • Mediastinal lymphadenopathy
  • Pulmonary fibrosis
  • Pulmonary nodules

Systemic symptoms

  • Fever
  • Fatigue
  • Weight loss

Liver (affecting around 20%)

  • Liver nodules
  • Cirrhosis
  • Cholestasis

Eyes (affecting around 20%)

  • Uveitis
  • Conjunctivitis
  • Optic neuritis

Skin (affecting around 15%)

  • Erythema nodosum (tender, red nodules on the shins caused by inflammation of the subcutaneous fat)
  • Lupus pernio (raised, purple skin lesions commonly on cheeks and nose)
  • Granulomas can develop in scar tissue

Heart (affecting around 5%)

  • Bundle branch block
  • Heart block
  • Myocardial muscle involvement

Kidneys (affecting around 5%)

  • Kidney stones
  • Nephrocalcinosis
  • Interstitial nephritis

Central nervous system (affecting around 5%)

  • Nodules
  • Pituitary involvement (diabetes insipidus)
  • Encephalopathy

Peripheral nervous system (affecting around 5%)

  • Bells palsy
  • Mononeuritis multiplex

Bones (affecting around 2%)

  • Arthralgia
  • Arthritis
  • Myopathy
187
Q

What is Lofgren’s Syndrome ?

A

This is a specific presentation of sarcoidosis. It is characteristic by a triad of:

  • Erythema nodosum
  • Bilateral hilar lympadenopathy
  • Polyarthralgia
188
Q

Differential diagnoses of sarcoidosis (5) ?

A
  • TB
  • Lymphoma
  • Hypersensitivity pneumonitis
  • Toxoplasmosis
  • Histoplasmosis
189
Q

Blood tests for sarcoidosis (five bullet points) ?

A
  • Raised serum ACE. This is often used as a screening test
  • Hypercalcaemia (raised calcium) is a key finding
  • Raised serum soluble interleukin-2 receptor
  • Raised CRP
  • Raised immunoglobulins
190
Q

Imaging for sarcoidosis (four bullet points) ?

A
  • CXR shows hilar lymphadenopathy
  • High resolution CT thorax shows hilar lymphadenopathy and pulmonary nodules
  • MRI can show CNS involvement
  • PET scan can show active inflammation in affected areas
191
Q

Gold standard test for confirming sarcoidosis + what would this test show if positive ?

A

Histology from biopsy. This is usually done by doing bronchoscopy with US guided biopsy of mediastinal lymph nodes.

The histology shows characteristic non-caseating granulomas with epitheloid cells.

192
Q

Tests for other organ involvement in sarcoidosis (6 bullet points) ?

A
  • U&Es for kidney involvement
  • Urine dipstick or urine albumin-creatinine ratio to look for proteinuria, indicating nephritis
  • LFTs for liver involvement
  • Opthalmology review for eye involvement
  • ECG and echocardiogram for heart involvement
  • US abdomen for liver and kidney involvement
193
Q

Treatment for sarcoidosis (four bullet points) ?

A
  • No treatment is considered as first line in pts with no or mild symptoms as it often resolves spontaneously
  • Oral steroids are usually first line where treatment is required and are given for between 6 and 24 months. Pts should be given biphosphonates to protect against osteoporosis whilst on such long term steroids
  • Second line options are methotrexate or azathioprine
  • Lung transplant is rarely required in severe pulmonary disease
194
Q

Prognosis of sarcoidosis ?

A

Sarcoidosis spontaneously resolves within 6 months in around 60% of pts. In a small number of pts it progresses and pulmonary fibrosis and pulmonary hypertension develop, potentially requiring a lung transplant. Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the CNS

195
Q

What causes obstructive sleep apnoea ?

A

Caused by collapse of the pharyngeal airway during sleep.

196
Q

Name 5 risk factors for obstructive sleep apnoea ?

A
  • Middle age
  • Male
  • Obesity
  • Alcohol
  • Smoking
197
Q

Name 7 features of obstructive sleep apnoea ?

A
  • Apnoea episodes during sleep (reported by their partner)
  • Snoring
  • Morning headache
  • Waking up unrefreshed from sleep
  • Daytime sleepiness
  • Concentration problems
  • Reduced oxygen saturation during sleep
198
Q

Name four complications of severe cases of obstructive sleep apnoea ?

A
  • HTN
  • Heart failure
  • Can increase risk of MI
  • Can increase risk of stroke
199
Q

What disease should you always suspect when someone presents with daytime sleepiness ?

A

Obstructive sleep apnoea

200
Q

Management of obstructive sleep apnoea ?

A
  • Referral to an ENT specialist or specialist sleep clinic where they can perform sleep studies. This involves the pt sleeping in a lab whilst staff monitor their oxygen sats, HR, RR and breathing to establish any apnoea episodes and the extent of their snoring
  • The first step in management is to correct reversible risk factors by advising them to stop drinking alcohol, stop smoking and loose weight.
  • The next step is to use a CPAP machine that provides pressure to maintain the patency of the airway
  • Surgery is another option. This involves quite significant surgical structuring of the soft palate and jaw. The most common procedure is called uvulopalatopharyngoplasty (UPPP).