Respiratory Flashcards
1
Q
What is alpha-1 antitrypsin deficiency?
A
Autosomal recessive inherited condition due to 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.
2
Q
What are the features of alpha-1 antitrypsin deficiency?
A
- 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
3
Q
What in investigations should be carried out in alpha-1 antitripsin deficiency?
A
A1AT concentrations
spirometry: obstructive picture
4
Q
What is the management for alpha-1 antitrypsin deficiency?
A
- No smoking
- supportive: bronchodilators, physiotherapy
- intravenous alpha1-antitrypsin protein concentrates
- surgery: lung volume reduction surgery, lung transplantation
5
Q
What is the mechanism of action of montelukast?
A
- leukotriene receptor antagonist.
- binds to the cysteinyl leukotriene receptor (CysLT1) in the bronchial tissue and blocking the action of cysteinyl leukotrienes, which are inflammatory mediators released during the immune response.
- leads to decreased inflammation, bronchoconstriction, mucus production and oedema in the airways, thus improving asthma symptoms.
6
Q
What are the features of bronchiolitis obliterans?
A
- progressive dyspnoea
- obstructive pattern on spirometry
- centrilobular nodules, bronchial wall thickening is seen on CT
- pulmonary complication of rheumatoid arthritis
7
Q
What are the features of allergic bronchopulmonary aspergillosis ?
A
- bronchoconstriction: wheeze, cough, dyspnoea. Patients may have a previous label of asthma
- bronchiectasis (proximal)
- eosinophilia
8
Q
What investigations are used in allergic bronchopulmonary aspergillosis?
A
- FBC: eosinophilia
- flitting CXR changes
- positive radioallergosorbent (RAST) test to Aspergillus
- positive IgG precipitins (not as positive as in aspergilloma)
- raised IgE
9
Q
What is the management for allergic bronchopulmonary aspergillosis?
A
Oral corticosteroids
itraconazole is sometimes introduced as a second-line agent
10
Q
What findings on spirometry indicate an obstructive picture?
A
FEV1 - significantly reduced
FVC - reduced or normal
FEV1% (FEV1/FVC) - reduced
11
Q
Which conditions cause an obstructive result on spirometry?
A
Asthma
COPD
Bronchiectasis
Bronchiolitis obliterans
12
Q
What findings suggest a restrictive picture on spirometry?
A
FEV1 - reduced
FVC - significantly reduced
FEV1% (FEV1/FVC) - normal or increased
13
Q
Which conditions cause a restrictive result on spirometry?
A
Pulmonary fibrosis
Asbestosis
Sarcoidosis
Acute respiratory distress syndrome
Infant respiratory distress syndrome
Kyphoscoliosis e.g. ankylosing spondylitis
Neuromuscular disorders
Severe obesity
14
Q
Which condition significantly reduced expiratory reserve volume?
A
Obesity
increased abdominal fat mass pushes up against the diaphragm, reducing the volume of air that can be expelled
15
Q
What are some causes of a raised transfer factor for carbon monoxide (TLCO)?
A
- asthma
- pulmonary haemorrhage (e.g. granulomatosis with polyangiitis, Goodpasture’s)
- left-to-right cardiac shunts
- polycythaemia
- hyperkinetic states
- male gender, exercise
16
Q
What are some causes of a lower TLCO?
A
- pulmonary fibrosis
- pneumonia
- pulmonary emboli
- pulmonary oedema
- emphysema
- anaemia
- low cardiac output
17
Q
What are the CXR stages of Sarcoidosis?
A
1 - bilateral hilar lymphadenopathy (BHL)
2 - BHL + infiltrates
3 - infiltrates
4 - fibrosis
18
Q
What are some potential investigation findings in sarcoidosis?
A
- spirometry: may show a restrictive defect
- tissue biopsy: non-caseating granulomas
- gallium-67 scan - not used routinely
- CXR changes
- Raised ESR
- Hypercalcaemia
- ACE
19
Q
Which organism most commonly causes bronchiectasis?
A
Haemophilus influenzae
20
Q
What is bronchiectasis?
A
permanent dilatation of the airways secondary to chronic infection or inflammation.
21
Q
What is the management for bronchiectasis?
A
- 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)
22
Q
What is the main role of alpha-1 antitrypsin in the body?
A
protease inhibitor used to protect the lungs from neutrophil elastase.
23
Q
What is the management of High Altitude pulmonary oedema (HAPE)?
A
descent
nifedipine, dexamethasone, acetazolamide, phosphodiesterase type V inhibitors*
oxygen if available
24
Q
What is extrinsic allergic alveolitis?
A
hypersensitivity induced lung damage due to a variety of inhaled organic particles
examples include:
- 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*
25
What are the features of extrinsic allergic alveolitis?
Acute:
- dyspnoea
- dry cough
- fever
Chronic (occurs weeks-months after exposure)
- lethargy
- dyspnoea
- productive cough
- anorexia and weight loss
26
What are the investigation findings of extrinsic allergic alveolitis?
- imaging: upper/mid-zone fibrosis
- bronchoalveolar lavage: lymphocytosis
- serologic assays for specific IgG antibodies
- blood: NO eosinophilia
27
What is the treatment for extrinsic allergic alveolitis?
Avoidance of precipitant
Oral corticosteroids
28
What are the features of small cell lung cancer?
- usually central
- arise from APUD* cells
- associated with ectopic ADH, ACTH secretion
ADH → hyponatraemia
ACTH → Cushing's syndrome
- ACTH secretion can cause bilateral adrenal hyperplasia, the high levels of cortisol can lead to hypokalaemic alkalosis
- Lambert-Eaton syndrome: antibodies to voltage gated calcium channels causing myasthenic like syndrome
29
What are the management options for small cell lung cancer?
- usually metastatic disease by time of diagnosis
- patients with very early stage disease (T1-2a, N0, M0) are now considered for surgery. NICE support this approach in their 2011 guidelines
however, most patients with limited disease receive a combination of chemotherapy and radiotherapy
patients with more extensive disease are offered palliative chemotherapy
30
What is idiopathic pulmonary fibrosis?
Chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs.
Unclear cause of the fibrosis.
31
What are the features of idiopathic pulmonary fibrosis?
progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations on auscultation
dry cough
clubbing
32
How is idiopathic pulmonary fibrosis diagnosed?
- 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
33
What are the indications for starting steroids in sarcoidosis?
- parenchymal lung disease
- uveitis
- hypercalcaemia
- neurological or cardiac involvement
34
What are common causes of respiratory alkalosis?
- anxiety leading to hyperventilation
- pulmonary embolism
- salicylate poisoning*
- CNS disorders: stroke, subarachnoid haemorrhage, encephalitis
- altitude
- pregnancy
35
Which factors prompt an assessment for LTOT in those with COPD?
- very severe airflow obstruction (FEV1 < 30% predicted). Assessment should be 'considered' for patients with severe airflow obstruction (FEV1 30-49% predicted)
- cyanosis
- polycythaemia (raised Hb)
- peripheral oedema
- raised jugular venous pressure
- oxygen saturations less than or equal to 92% on room air
36
What suggests a chest drain should be inserted in a patient with pleural effusion secondary to infection?
- if the fluid is purulent or turbid/cloudy a chest tube should be placed to allow drainage
- if the fluid is clear but the pH is less than 7.2 in patients with suspected pleural infection a chest tube should be placed
37
What are some causes of upper zone fibrosis?
- hypersensitivity pneumonitis (also known as extrinsic allergic alveolitis)
- coal worker's - pneumoconiosis/progressive massive fibrosis
- silicosis
- sarcoidosis
- ankylosing spondylitis (rare)
- histiocytosis
- tuberculosis
- radiation-induced pulmonary fibrosis
38
What is the investigation of choice for upper airway compression?
Flow volume loop
39
What is granulomatosis polyangiitis?
- autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.
- New name for Wegener's granulomatosis
40
What are the features of Granulomatosis with polyangiitis?
- 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
41
What are the investigation findings for granulomatosis with polyangiitis?
- 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
42
What is the management for granulomatosis with polyangiitis?
steroids
cyclophosphamide (90% response)
plasma exchange
median survival = 8-9 years
43
What are the landmarks of the triangle of safety for chest drains?
base of the axilla
lateral edge pectoralis major
5th intercostal space
anterior border of latissimus dorsi
44
What are the starting setting when commencing NIV (BiPAP)?
IPAP 10cm H20
EPAP 5cm H20
45
What is the first line investigation for suspected asthma?
- measure the eosinophil count OR fractional nitric oxide (FeNO)
- diagnose asthma, without further investigations, if:
* eosinophil is above the reference range
* FeNO is ≥ 50 ppb
46
What paraneoplastic features are associated with squamous cell lung cancer?
- parathyroid hormone-related protein (PTH-rp) secretion causing hypercalcaemia
- clubbing
- hypertrophic pulmonary osteoarthropathy (HPOA)
- hyperthyroidism due to ectopic TSH
47
What are the contraindications to surgery in non small cell lung cancer?
- assess general health
- stage IIIb or IV (i.e. metastases present)
- FEV1 < 1.5 litres is considered a general cut-off point*
- malignant pleural effusion
- tumour near hilum
- vocal cord paralysis
- SVC obstruction
48
Which cancers are associated with calcification of lung metastases?
osteosarcomas
papillary thyroid carcinoma
chondrosarcoma
49
What are the most common presenting features in cystic fibrosis?
- neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice
- recurrent chest infections (40%)
- malabsorption (30%): steatorrhoea, failure to thrive
- other features (10%): liver disease
50
What are some other features of CF?
- short stature
- diabetes mellitus
- delayed puberty
- rectal prolapse (due to bulky stools)
- nasal polyps
- male infertility, female subfertility
51
What is Lights criteria for determining if an exudate effusion?
- Pleural fluid protein / Serum protein >0.5
- Pleural fluid LDH / Serum LDH >0.6
- Pleural fluid LDH > 2/3 * Serum LDH upper limit of normal
52
What is catamenial pneumothorax?
Spontaneous PTX occurring 72hrs before or after onset of menstruation
53
What is Kartagener's syndrome?
- primary ciliary dyskinesia association with dextrocardia
- dynein arm defect results in immotile cilia
54
What are the features of Kartagener's syndrome?
- dextrocardia or complete situs inversus
- bronchiectasis
- recurrent sinusitis
- subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes)
55
What is Heerfordt syndrome?
parotid enlargement, fever and uveitis secondary to sarcoidosis
56
What is the pathophysiological mechanism of emphysema?
Destruction of alveolar walls secondary to proteinases
Results in decreased elastic recoil, air trapping, and reduced gas exchange surface area.
57
Causes of transudate effusion (protein <30g/L)?
- heart failure (most common transudate cause)
- hypoalbuminaemia
- liver disease
- nephrotic syndrome
- malabsorption
- hypothyroidism
- Meigs' syndrome
58
What are causes of exudate effusion (protein >30g/L)?
- infection
* pneumonia (most common exudate cause),
* tuberculosis
* subphrenic abscess
- connective tissue disease
* rheumatoid arthritis
* systemic lupus erythematosus
- neoplasia
* lung cancer
* mesothelioma
* metastases
- pancreatitis
- pulmonary embolism
- Dressler's syndrome
- yellow nail syndrome
59
What does a normal pCO2 in acute asthma attack suggest?
Life threatening asthma
60
Which criteria demonstrate life threatening asthma?
- PEFR < 33% best or predicted
- Oxygen sats < 92%
- 'Normal' pC02 (4.6-6.0 kPa)
- Silent chest, cyanosis or feeble respiratory effort
- Bradycardia, dysrhythmia or hypotension
- Exhaustion, confusion or coma
61
What is silicosis?
fibrosing lung disease caused by the inhalation of fine particles of crystalline silicon dioxide (silica).
It is a risk factor for developing tuberculosis
62
Which occupations are at risk of silicosis?
mining
slate works
foundries
potteries
63
What are the features of silicosis?
- upper zone fibrosing lung disease
- 'egg-shell' calcification of the hilar lymph nodes
64
What is the 1st line treatment for COPD?
Short acting beta 2 agonist e.g. salbutamol inhaler or Short acting muscarinic antagonist.
65
What features suggest asthma/steroid responsive in COPD?
- any previous, secure diagnosis of asthma or of atopy
- a higher blood eosinophil count
- substantial variation in FEV1 over time (at least 400 ml)
- substantial diurnal variation in peak expiratory flow (at least 20%)
66
What is 2nd line treatment in COPD with no asthmatic/steroid responsive features?
- add a long-acting beta2-agonist (LABA) + long-acting muscarinic antagonist (LAMA)
if already taking a SAMA, discontinue and switch to a SABA
67
What is the 2nd line treatment for COPD with asthmatic/steroid responsive features?
LABA + inhaled corticosteroid (ICS)
68
What is the 3rd line treatment for COPD with asthmatic/steroid responsive features?
triple therapy i.e. LAMA + LABA + ICS
if already taking a SAMA, discontinue and switch to a SABA
69
What are predisposing factors to obstructive sleep apnoea?
obesity
macroglossia: acromegaly, hypothyroidism, amyloidosis
large tonsils
Marfan's syndrome
70
What are the features of lung carcinoid?
majority of bronchial adenomas are carcinoid tumours
- typical age = 40-50 years
- smoking not risk factor
- slow growing: e.g. long history of cough, recurrent haemoptysis
- often centrally located and not seen on CXR
- 'cherry red ball' often seen on bronchoscopy
- carcinoid syndrome itself is rare (associated with liver metastases)
71
What is the management of lung carcinoid?
Surgical resection
90% survival at 5 years if no mets
72
On which chromosome is the cystic fibrosis transmembrane regulator gene?
Chromosome 7
73
Which medication can be used to prevent acute mountain sickness?
Acetazolamide (a carbonic anhydrase inhibitor)
74
What is the management of Cystic Fibrosis?
- regular (at least twice daily) chest physiotherapy and postural drainage.
- high calorie diet, including high fat intake
- pancreatic enzymes with every meal if insufficient production
- minimise contact with other CF patients to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa
- vitamin supplementation
- lung transplantion
75
What is a contraindication to lung transplant in CF?
chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation
76
What medication can be used to treat CF?
Lumacaftor/Ivacaftor (Orkambi) used to treat those who are homozygous for the delta F508 mutation
- lumacaftor increases the number of CFTR proteins that are transported to the cell surface
- ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore
77
Which gene is associated with bronchiectasis?
HLA-DR1
78
What are some key indications for NIV?
- COPD with respiratory acidosis pH 7.25-7.35
-T2RF secondary to chest wall deformity, neuromuscular disease or obstructive sleep apnoea
- cardiogenic pulmonary oedema unresponsive to CPAP
- weaning from tracheal intubation
79
What are high risk characteristics in pneumothorax that indicate need for chest drain insertion?
- Haemodynamic compromise (suggesting a tension pneumothorax)
- Significant hypoxia
- Bilateral pneumothorax
- Underlying lung disease
- ≥ 50 years of age with significant smoking history
- Haemothorax
80
What is the management of high altitude cerebral oedema?
Decent
Dexamethasone
81
What tests are diagnostic for obstructive sleep apnoea?
polysomnography (sleep studies)
82
Which conditions predominantly cause lower zone fibrosis?
- idiopathic pulmonary fibrosis
- most connective tissue disorders (except ankylosing spondylitis) e.g. SLE
- drug-induced: amiodarone, bleomycin, methotrexate
- asbestosis
83
