WEEK 3 - chronic breathlessness Flashcards
sympathetic innervation of bronchiolar smooth muscle is mediated by ______ acting on ________ to cause ________.
parasympathetic innervation of bronchiolar smooth muscle is mediated by _______ acting on ______ to cause ________.
- noradrenaline
- beta receptors
- bronchodilation
- acetylcholine
- muscarinic receptors
- bronchoconstriction
type I vs type II alveolar cells
type I
- gas exchange between the alveoli and capillaries
- larger squamous (flattened) cells
- no secretory organelles present
- less numerous than type II
type II
- secrete surfactant to lower surface tension
- smaller cuboid-shaped cells
- secretory organelles present
- more numerous than type I
the lungs have dual arterial supply and venous drainage, comprised of the ________ arteries and veins, and the __________ arteries and veins.
the pulmonary arteries supply _________ from the ______ to the _____ capillary network. the pulmonary veins drain _______ blood to the _________.
the bronchial artiereis supply ________ blood from the ________ to the lung tissues. the bronchial veins drain ________ blood to the pulmonary and systemic venom systems
- pulmonary
- bronchial
- deoxygenated
- right ventricle
- alveolar
- oxygenated
- left atrium
- oxygenated
- thoracic aorta
- deoxygenated
what is the MRC (medical research council) dyspnoea scale?
a scale to make an objective assessment of the symptom of breathlessness when taking a history
breathlessness when lying flat is a key symptom of what?
congestive cardiac failure (CCF) (orthopnoea)
bilateral vs unilateral ankle swelling
bilateral — sign of cardiac pathology, esp CCF
unilateral — may indicate DVT/PE
what classically can cause breathlessness with lightheaded mess?
aortic stenosis
why ask about exposure to birds?
hypersensitivity pneumonitis
a type of hypersensitivity pneumpnitis due secondary to repeated inhalation of avian antigens is seen in bird keeps
sometimes known as ‘Pigeon Fancier’s Lung’
what are the hallmark symptoms of COPD?
- SOB
- chronic cough
- sputum production
other features:
- winter exacerbations
- wheeze
what are the physical signs of COPD?
- accessory muscle use
- auscultation
- chest hyperexpansion
- chest wall movement
- tar-staining
- peripheral oedema
- palpable liver
- pursed lip breathing
explain pursed lip breathing
this enables a patient to reduce their resp rate by increasing their period of expiration; it creates resistance to expiratory airflow and development of a positive expiratory pressure in the airways, reducing airway collapse and aiding ventilation
in COPD, what impede the effective chest expansion by the diaphragm and intercostal muscles? what does the patient do instead?
air trapping and hyperinflation
instead the patient used accessory muscles (inc SCM, scalene, trapezius and abdominal muscles) to aid ventilation
what does peripheral oedema in COPD indicate?
right-sided heart failure due to cor pulmonale
what is cor pulmonale?
abnormal enlargement of the right side of the heart as a result of disease of the lungs or the pulmonary blood vessels
why might you get a palpable liver in COPD?
may be due to hyperinflation of the lungs or congestive heart failure
what would you hear in auscultation of COPD?
- reduced breath sounds
- reduced heart sounds
- tachypnoea
- wheeze
during an exacerbation there may also be crepitations
describe chest wall movement in COPD
- reduced lateral (bucket handle) chest expansion
- increased vertical (pump handle) chest expansion
what rare genetic condition can cause COPD at a young age, esp in smokers?
alpha-1 anti trypsin deficiency
what is the gold standard investigation for diagnosing COPD and grading COPD severity?
spirometry
what are 3 histological features of COPD? what symptoms do they cause?
- goblet cell hyperplasia — cough and sputum
- airway narrowing — breathlessness and wheeze
- alveolar destruction — breathlessness
inflammation mediated by the toxic substances of tobacco smoke cause these things
how does NICE define airflow obstruction?
post-bronchodilator FEV1/FVC ratio such that FEV1/FVC is less than 0.7
(above 70% is normal)
FEV1/FVC obstructive vs restrictive disease
O = <75%
R = > 75% (FEV1 and FVX are reduced in roughly the same proportion so the ratio remains within the normal range)
premature airway ______ is a key feature of COPD
collapse
list some restrictive vs obstructive disorders
restrictive — pulmonary fibrosis, sarcoidosis., chest wall deformity, and neuromuscular disorders
obstructive — COPD, asthma, bronchiectasis, cystic fibrosis
describe COPD natural history
- progressive decline in lung function
- progressive dyspnoea and disability
- right ventricular failure (cor pulmonale)
- exacerbations become more frequent and contribute to morbidity and disability
describe hypoxia to RV failure
hypoxia —> pulmonary artery vasoconstriction —> increased pulmonary artery pressure —> right ventricular hypertrophy —> right ventricular failure
what are the fundamentals of COPD care?
- treatment and support to stop smoking
- vaccinations — influenza and pneumococcal
- physiotherapy/pulmonary rehabilitation if indicated
- co-develop a personalised self-management plan
- optimise treatment for co-morbidities
what is the best combination to achieve smoking cessation?
champix combined with NRT and behavioural support
what is champix?
varenicline
nicotine receptor partial agonist
for COPD, inhaled therapies should only be offered if what?
- needed to relieve SOB and exercise limitation
- patient has been trained to use inhalers and has satisfactory technique
what is the most common short-acting muscarinic antagonist?
ipratropium
what is step 1 of COPD treatment?
offer SABA or SAMA
what is step 2 of COPD treatment if no asthmatic features (previous secure diagnosis of asthma or autopsy, raised blood eosinophil counts substantial variation in FEV1 over time pr diurnal variation in PEF) ?
offer DUAL therapy : LABA + LAMA
LABA eg. formoterol, salmeterol
LAMA eg. tiotropium, glucopyrronium
what is step 2 of COPD treatment if asthmatic features present?
DUAL therapy with LABA + inhaled corticosteroids (ICS)
ICS eg. beclomethasone — brown inhaler
salmeterol with fluticasone — purple inhaler
what is step 3 of COPD treatment? (continue to have day-to-day symptoms that adversely impact QoL, OR have 1 severe or 2 moderate exacerbations within a year, even on dual therapy)
triple therapy : LABA + LAMA + ICS
who is home oxygen therapy for in COPD?
patients with a paO2 < 7.3 on air
or paO2 7.3-8.0 on air and either:
- secondary polycythaemia
- peripheral oedema
- pulmonary hypertension
only available to NON-SMOKING HOUSEHOLDS
magnesium in asthma/COPD?
bronchodilator typically used for treating acute exacerbations of asthma, and rarely used in acute COPD exacerbations
long-acting muscarinic antagonists are used in management of ____ but not ____
COPD but not asthma
brand names for inhalers?!
surely not
what are the side effects of inhaled corticosteroid therapy?
inhaled corticosteroids generally have less systemic absorption and therefore less systemic side effects than oral corticosteroids, and the main side effects are candidiasis and hoarseness.
however, with prolonged use at higher doses you may see side effects of adrenal suppression, osteoporosis and growth restriction in children
COPD exacerbation treatment
- oxygen
- high dose SABAs, usually nebulised
- high dose corticosteroids (usually prednisone 40mg/day — 7 days)
- antibiotic only if purulent sputum or very severe illness
- reassess after 1 hr
if after 1 hour still respiratory acidosis, consider all of:
- iv bronchodilator (salbutamol or theophylline)
- urgent intensive care opinion
- non-invasive ventilation
- intubation and assisted ventilation
What is the pathological process that leads to Type II respiratory failure in COPD? What physiological compensatory mechanisms occur in the body to reduce the level of acidaemia?
In COPD the elastic recoil of the lungs is lost. This causes gas trapping and reduced excretion of carbon dioxide. Sputum retention also means there is less surface area for gas exchange. In the blood, the carbon dioxide combines with water to form carbonic acid.
In an acute setting, the increased acid levels in the blood would lower the pH levels and the patient would become unwell very quickly. However, when carbon dioxide retention is slowly progressive, as is often the case in chronic COPD, the body can compensate for this by utilising the bicarbonate buffer system of the blood. The kidneys are stimulated to reabsorb more bicarbonate, which acts as a base and neutralises the carbonic acid, thus restoring the pH back to the normal range.
hypercapnia causes what type of pulse?
bounding pulse and tachycardia
eyes in hypercapnia?
dilated pupils and papilloedema
what can been seen in this x ray that might suggest COPD?
- flattened diaphragms
- nipple shadows
- smaller heart size
- hyper inflated lung
- horizontal ribs
what respiratory conditions are associated with nail clubbing?
lung cancer, suppurative lung diseases (bronchiectasis, CF), and interstitial lung diseases
FEV1, FVC + FEV1/FVC in interstitial lung disease
In ILD a restrictive pattern of lung function is seen. FVC and FEV1 are low but proportionally so, so that the FVC/FEV1 ratio remains normal. Lung volumes are also reduced, as is the transfer factor, which measures the lungs’ ability to ‘soak up’ oxygen.
ILD is an umbrella term for over a hundred different conditions affecting the lung _________
parenchyma
Different forms ILD can be categorised as primarily _______________ or primarily _________
- inflammatory
- fibrotic
the inflammatory conditions my eventually lead to fibrosis if left unchecked. distinguishing between the two is important with respect to the treatments available
what forms of ILD is smoking associated with?
Desquamative Interstitial Pneumonia (DIP) and Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)
what kind of crepitations are heard in fibrotic lung disease?
bi-basal, fine, end-inspiration crepitations are a pathognomonic feature of fibrotic lung disease
somestimes described as ‘velcro-like’
what are some unknown causes of ILD?
- idiopathic pulmonary fibrosis
- sarcoidosis
- cryptogenic organising pneumonia
what are some known causes of ILD?
- occupational : asbestosis/silicosis
- environmental : hypersensitivity pneumonities (pigeons/birds)
- immune : Connective Tissue Disease (RA; systemic sclerosis)
- smoking : DIP and RB-ILD
- drugs : any!
fibrotic vs inflammatory causes of ILD?
inflammatory eventually turn into fibrotic if left unchecked
what are symptoms and signs of connective tissue disease?
Raynaud’s, swollen/stiff joints, dry eyes, dry mouth, mouth ulcers, photosensitive rash, skin changes, sclerodactyly, telangiectasia, periungual erythema, dysphagia
methotrexate is an anti-inflammatory known to cause ILD in what form?
hypersensitivity pneumonitits
amiodarone is an _______ medication known to cause ILD leading to ________
- anti-arrhythmic
- lung fibrosis
many chemotherapy agents can cause ILD leading to lung fibrosis, but ________ is one of the agents most commonly associated with it
bleomycin
what is an antibiotic known to cause ILD leading to lung fibrosis with long-term use )eg. when sued for UTI prophylaxis)?
nitrofurantoin
name 2 anti fibrotics used to slow down the progression of ILD
Pirfenidone and Nintedanib
what are the common SEs of anti fibrotics?
gastrointestinal — loss of appetite, nausea, reflux
pirfenidone — also lethargy and can get a skin rash (so must wear factor 50 sun cream all the time)
nintedanib — loose stool and diarrhoea
IPF prognosis?
very poor prognosis despite optimal intervention with ant-fibrotic medication
- mean survival time after diagnosis is 2.5 to 5 years without treatment
- 2-3 years longer with antifibrotic therapy
although the natural history of the disease varies greatly between patients, the general trend is progressive worsening of symptoms, although the speed at which this happens is highly variable
history of weight loss, fever and night sweats, with a CXR showing bifilar lymphadenopathy are indicative of what 3 possible diagnosis?
- sarcoidosis
- tuberculosis
- lymphoma
what is acid-fast bacillus?
a type of bacteria that causes TB
what are the 3 key presenting features of ILD?
- SOB on exertion
- dry cough
- fatigue
what are 2 typical findings on examination for idiopathic pulmonary fibrosis?
- bibasal fine end-inspiratory crackles
- finger clubbing
what is asbestosis?
lung fibrosis related to asbestos exposure
what is sarcoidosis?
a chronic granulomatous disorder
granulomas are inflammatory nodules full of macrophages. the cause of these granulomas is unknown. it is usually associated with respiratory symptoms but has many extra-pulmonary manifestations, such as erythema nodosum and lymphadenopathy. symptoms can vary dramatically from asymptomatic to severe or life-threatening
Sarcoidosis can affect anyone but seems to be slightly more common in who?
- aged 20-39 or around 60
- women
- black ethnic origin
what are some skin features of sarcoidosis?
less than half of patients have skin involvement
- erythema nodosum — characterised by nodules of inflamed subcutaneous fat on the shins. presents as raised, red, tender, painful, subcutaneous nodules across both shins. over time the nodules settle and appear as bruises. there are many causes of erythema nodosum.
- lupus pernio is specific to sarcoidosis and presents with raised purple skin lesions, often on the cheeks and nose.
how does sarcoidosis present in the lungs?
- mediastinal lymphadenopathy
- pulmonary fibrosis
- pulmonary nodules
how does sarcoidosis present in the liver?
- liver nodules
- cirrhosis
- cholestasis
how can sarcoidosis present in the eyes?
- uveitis
- conjunctivitis
- optic neuritis
how can sarcoidosis present in the heart?
- bundle branch block
- heart block
- myocardial muscle involvement
how can sarcoidosis present in the kidneys?
- kidney stones (due to hypercalcaemia)
- nephrocalcinosis
- interstitial nephritis
how can sarcoidosis present in the central and peripheral nervous system?
central:
- nodules
- pituitary involvement (diabetes insipidus)
- encephalopathy
peripheral:
- facial nerve palsy
- mononueritis multiplex
how can sarcoidosis present in bones?
- arthralgia
- arthritis
- myopathy
Lofgren’s syndrome (acute form of sarcoid) refers to a specific presentation of sarcoidosis with a classic triad of what symptoms?
- erythema nodosum
- bilateral hilar lymphadenopathy
- polyarthralgia (joint pain in multiple joints)
and fever
treatment of Lofgren syndrome
will settle with NSAIDS — see back in 3 months with repeat CXR
describe the staging of pulmonary sarcoidosis
stage 0 = normal chest radiograph
stage 1 = hilar adenopathy alone
stage 2 = hilar adenopathy with parenchymal involvement
stage 3 = parenchymal involvement alone
stage 4 = pulmonary fibrosis
1 + 2 may resolve on their own without treatment
sarcoidosis laboratory tests
- leukopenia
- elevated ESR
- hypercalcaemia in 5%
- hypercalciruia in 20%
- ACE levels — commonly elevated in active disease. neither sensitive nor specific enough to be of diagnostic value
what does a transbronchial biopsy show in sarcoidosis?
- noncaseating granuloma
- has a yield of 75-90%
- biopsy may be unnecessary in stage 1 disease with a presentation highly suggestive of sarcoidosis
sarcoidosis: pulmonary function tests may show _______ or ________, with diminished ______ capacity
- obstruction
- restriction
- diffusion
sarcoidosis:
bronchoalveolar lavage: increase in lymphocytes with a high ________ ratio; used to follow disease activity, but not for diagnosis
CD4:CD8
Why is it important to wean steroid treatment slowly rather than finish a course abruptly?
Administering exogenous steroids causes suppression of endogenous cortisol production by the adrenal glands. Abrupt withdrawal of exogenous steroids can therefore precipitate an adrenal crisis. A gradual reduction in exogenous steroid dose enables the adrenal glands to resume their normal function.
what is given for moderate to severe sarcoidosis?
0.5mg per kg prednisone 2-4 weeks
taper quickly according to respsone
maintenance: slow taper 1mg/month
if relapse during wean — consider adding methotrexate
what are tram track opacities?
dilated airways seen in the longitudinal plane
what are ring opacities?
dilated airways seen end on
what does mucus plugging of airways result in?
airway dilatation
an X ray alone is insufficient to definitively diagnose bronchiectasis. ______________ is the diagnostic imaging of choice and will also help to exclude alternative differentials such as lung cance
high resolution CT scan
bronchiectasis is a chronic condition in which the bronchial tree becomes abnormally and irreversibly ________. The bronchial walls become _________ and _________, leading to a build-up of excess mucus which can make the lungs vulnerable to ________.
- dilated
- thickened
- inflamed
- infection
patients typically present with recurrent chest infections, persistent cough with sputum production, and shortness of breath.
what is an inherited form of bronchiectasis? it is caused by defects in what gene?
cystic fibrosis (CF) in CFTR gene
what are the causes of bronchiectasis?
- Idiopathic (most common)
- Post-infective – e.g., bacterial pneumonia, mycobacterial infection
= Allergic – e.g., to mould or fungus, especially in allergic bronchopulmonary aspergillosis (ABPA) - Autoimmune – associations with connective tissue diseases and inflammatory bowel disease
- Immunodeficiency – e.g., primary ciliary dyskinesia, primary immunodeficiency disorders, HIV/AIDs
- Obstruction – secondary to severe asthma or COPD, or distal to a neoplasm or inhaled foreign body
what is the screening test of choice for suspected CF? what is the definitive diagnostic test for CF performed if the first test is done and indicative of CF?
- sweat test
- then CFTR genetic testing
what is usually required if bronchiectasis secondary to obstruction by a neoplasm is suspected?
bronchoscopy
what is ABPA?
allergic bronchopulmonary aspergillosis
a fungal infection of the lung due to a hypersensitivity reaction to antigens of Aspergillus fumigatus after colonization into the airways. Predominantly it affects patients with bronchial asthma and those having cystic fibrosis.
how is ABPA diagnosed?
certain clinical characteristics and the presence of elevated total IgE
bronchiectasis treatment
- Physiotherapy and airway clearance techniques
- Cough assist devices
- Smoking cessation
- Pneumococcal and annual influenza vaccinations
- Treatment with short courses of antibiotics for exacerbations or chest infections
- In rare cases surgery, for example a lobectomy if bronchiectasis is localised and symptoms are debilitating
what is an exacerbation of bronchiectasis?
deterioration in three or more of the following key respiratory symptoms for at least 48 hours:
- Cough
- Sputum volume and/or consistency
- Sputum purulence
- Breathlessness and/or exercise tolerance
- Fatigue and/or malaise
- Haemoptysis
They are not always the result of acute infection, and antibiotics are not always indicated.
what are some uncommon complications and a rare but serious complication of bronchiectasis?
Uncommon complications are the development of pulmonary hypertension and cor pulmonale. A rare but serious complication of bronchiectasis is massive haemoptysis.
salmeterol inhaler and tiotropium inhaler
uncompensated type II respiratory failure
A 58 y/o man presents with an acute exacerbation of COPD. Treatment is nebulized salbutamol, ipratropium and IV hydrocortisone. There is minimal improvement and IV aminophylline is commenced. What is the mechanism of action of Aminophylline?
phosphodiesterase inhibitor
hyper-resonant percussion note, polyphonic wheeze
PaO2 7.2kPa
bronchoscopy with biopsy EBUS
bronchiectasis
honeycombing
nitrofurantoin
