Respiratory Disease Profiles Flashcards
1
Q
central tolerance
A
deletion of self-reactive lymphocytes in primary lymphoid tissues
2
Q
peripheral tolerance
A
inactivation of self-reactive lymphocytes in peripheral tissues that escapes central tolerance, eg by TREG cells
3
Q
IPEX syndrome
A
X linked mutation in the FOXP3 gene (involved in TREG development) autoimmune disease
4
Q
what alleles have been identified which predispose individuals to autoimmune diseases
A
HLA alleles
5
Q
type I hypersensitivity reaction
A
immediate hypersensitivity, onset in seconds, IgE mediated antibody response to external antigen. asthma, hayfever, anaphylaxis
6
Q
sensation stage of type 1 hypersensitivity reaction
A
production of specific IgE by B cells in response to initial allergen exposure, residual IgE antibodies bind to circulating mast cells via Fc receptors
7
Q
allergic stage of type 1 hypersensitivity reaction
A
on re-exposure to allergen, allergen will bind to IgE coated mast cells -> cell degranulation, release of histamine + inflammatory mediators
8
Q
type II hypersensitivity reaction
A
onset is seconds/hours, IgM/IgG antibodies bind to antigens on cells of particular tissue types -> complement system activation, or antibody dependent cell-mediated cytotoxicity. goodpastures syndrome, graves disease
9
Q
type III hypersensitivity reaction
A
onset is hours, antibody binds to excess soluble antigen (in circulation) producing small immune complexes which are trapped in small blood vessels, joints and glomeruli -> activate complex -> attracts inflammatory cells.
hypersensitivity pneumonitis, SLE
10
Q
type IV hypersensitivity reaction
A
onset is days, T cell mediated, release of inflammatory cytokines and cell mediated cytotoxicity.
TB, sarcoidosis, rheumatoid arthritis
11
Q
primary immunodeficiency disorders
A
part of immune system missing/does not function properly, due to genetic causes
12
Q
clinical presentation of primary immunodeficiency disorders
A
weight loss, eczema, chronic diarrhoea, mouth ulceration, SPUR infections (serious, persistent, unusual, reccurent)
13
Q
severe congenital neutropenia
A
low neutrophils, type I is defect in gene that codes for neutrophil elastase, defect in neutrophil development
14
Q
leukocyte adhesion deficiency
A
defect in CD18 integrin gene, neutrophils cannot recognise markers expressed on endothelial cells, failure of neutrophil adhesion and migration
15
Q
chronic granulomatous disease
A
inability of macrophages to generate oxygen/nitrogen free radicals -> impaired killing of intracellular microorganisms -> excessive inflammation -> granuloma
16
Q
severe combined immunodeficiency
A
failure to produce lymphocytes
17
Q
x-linked SCID
A
mutation of IL-2 receptor -> cant respond to cytokines -> failure of T and NK cell development, production of immature B cells
18
Q
bruton’s x-linked hypogammaglobulinaemia
A
mutation in BTK gene, essential for B cell development
19
Q
asthma
A
chronic inflammatory condition of the airways that causes episodic exacerbations of bronchoconstriction
20
Q
sympathetic and parasympathetic controls of bronchi
A
sympathetic- bronchodilation, decreases mucous secretion via B2 adrenoreceptors
parasympathetic- bronchoconstriction, increases mucous secretion via M3 receptors
21
Q
non-atopic asthma
A
low level TH1 response to antigen
triggers- infection, night time/early morning, exercise, animal dander, cold, dust, strong emotions
IgG and macrophages
22
Q
atopic asthma
A
strong TH2 response to antigen
mast cells (initial asthma attack), eosinophil accumulation (late phase)
23
Q
key characteristics of asthma
A
reversible airflow obstruction via M3 receptors (parasympathetic)
bronchial inflammation
bronchial hyperresponsiveness (caused by damage to epithelium)
over time, airway remodeling (increased goblet cells and smooth muscle)
24
Q
signs and symptoms
A
episodic symptoms, diurnal variability, dry cough with wheeze + SoB, history of other atopic conditions, family history
25
spirometry testing for asthma results
obstructive pattern, decreased FEV1, FVC normal -> reduced FEV1/FVC ratio
>15% improvement in FEV1/PEFR following inhalation of a bronchodilator
26
long term management of asthma
SABA + ICS
SABA + ICS + LABA/LAMA
add LTRA/theo/chromone
add oral steroid and anti-IgE/anti-IL5/anti-IL4a
27
management of acute presentation of asthma
OSHITMAn- oxygen, salbutamol, hydrocortisone (IV)/oral prednisolone, Ipratropium, Theophylline, magnesium sulphate, an anesthetist (to intubate)
28
COPD
lung disease characterised by airway obstruction due to inflammation of the small airways, caused predominantly by inhaled toxins, especially via smoking
29
pathophysiology of chronic bronchitis
cigarette smoke -> chronic neutrophil inflammation -> scarring and fibrosis
hypertrophy of mucus secreting glands and hyperplasia of goblet cells
30
pathophysiology of emphysema
inflammation -> neutrophils release proteases -> break down elastin walls of alveoli -> loss of elastic recoil (abnormally increased compliance)
31
signs and symptoms of COPD
progressive dyspnoea, chronic cough, regular exacerbations, wheezing (chronic bronchitis), reduced breath sounds (emphysema)
32
diagnosis of COPD- spirometry and DLCO
obstructive pattern- decreased FEV1, FVC normal -> reduced FEV1/FVC ratio
DLCO decreased in emphysema
33
long term management of COPD
>2 exacerbations + large impact on life- LAMA. LAMA + LABA, ICS + LABA
>2 exacerbations- LAMA
0/1 exacerbations + large impact on life- LABA or LAMA
0/1 exacerbations- bronchodilator
33
hospital management of an exacerbation
ISOAP- ipratropium, salbutamol, oxygen, amoxicillin, prednisolone
34
obstructive sleep apnoea
pauses in respiration during sleep, as a result of upper airway collapse
35
causes of sleep apnoea
obesity, enlarged tonsils/adenoids, retrognathia, acromegaly, hyperthyroidism, neurological (stroke), anaesthesia (post op)
36
pathophysiology of sleep apnoea
airway at back of throat sucked closed when breathing in -> hypoxia + increasingly strenuous respiratory efforts until patient overcomes the resistance -> patient wakes up (awakening so brief, may be unaware)
37
management of sleep apnoea
CPAP, mandibular advancement device for mild OSA, surgery (mandibular advancement surgery)
38
cystic fibrosis
genetic disease, abnormally viscous mucus, blocks airways and lungs, results in repeated chest infections and chronic colonisation
39
what causes CF
mutation involving the material that codes for the cystic fibrosis transmembrane regulatory (CFTR) protein
40
pathophysiology of CF
CFTR protein normally forms channel on mucosal surfaces that allow chloride ions out of the cell and into the lumen. Na normally follows Cl so Na secretion also reduced, less Na in lumen = increased water reabsorption from lumen into epithelial cells, results in viscous mucus that blocks ducts + impairs mucosal defense
41
respiratory symptoms of CF
recurrent resp infections, chronic daily cough + sputum production, dyspnoea, nasal polyps, haemoptysis
42
other symptoms of CF
salty sweat, infertility in males (bilateral absence of vas deferens), CF-related diabetes
43
CFTR modulators
improve flow through CFTR- ivacaftor, orkambi, symkevi, trikefta
44
bronchiectasis
irreversible and abnormal dilation of the bronchial tree, caused by cycles of bronchial inflammation, mucus plugging and progressive airway destruction
45
causes of bronchiectasis
CF, bronchial narrowing/obstruction (COPD, aspiration, tumours, ABPA), lung infection (pneumonia, TB), immunodeficiency
46
pathophysiology of bronchiectasis
excessive inflammatory response to infection/obstruction -> fibrosis, airway dilates as surrounding scar tissue contracts, dilation allows for stasis of mucus -> chronic infection
47
most common bacteria involved in bronchiectasis
haemophilus influenzae and pseudomonas aerginosa
48
signs and symptoms of bronchiectasis
chronic productive cough, fever + malaise, haemoptysis, clubbing, reccurent infections, coarse crackles
49
what does a CT show in bronchiectasis
thickened and dilated airways
50
common cold
acute viral infection of the nasal passages, most commonly rhinovirus, may be coronavirus or RSV
51
croup
common childhood illness caused by inflammation of the URT as a result of viral infection
52
common viruses causing croup
parainfluenza virus I, II,III or IV, RSV, adenovirus
53
pathophysiology of croup
URTI causes nasopharyngeal inflammation that may spread to larynx + trachea, causing subglottal inflammation, oedema + compromise of airway at its narrowest portion
54
clinical presentation of croup
barking cough, hoarseness, stridor, symptoms worse at night
55
diptheria
life threatening bacterial throat infection, characteristic pseudo-membrane, vaccinated against in UK
56
organisms causing epiglottitis
streptococcus spp. staph aureus and pseudomonass spp
57
clinical presentation of epiglottitis
sore throat, odynophagia (painful swallowing), inability to swallow secretions (drooling in children), muffled voice, fever
58
antibiotic used in treatment of epiglottitis
ceftriaxone
59
causes of tonsillitis
viral- EBV, rhinovirus, influenza, parainfluenza, enterovirus, adenovirus
bacterial- strep pyrogenes, H influenza, staph aureus, strep pneumonia
60
complications of tonsillitis
otitis media, peritonsillar abscess, parapharyngeal abscess, epiglottitis
61
rhinitis
acute or chronic inflammation of the nasal mucosa
62
causes of rhinitis
allergen, infection, hormonal imbalance, medications
63
pathophysiology of allergic rhinitis
inhalation of allergen increases specific IgE levels, IgE binds to receptors on mast cells + basophils, re-exposure causes degranulation of mast cells + basophils
64
clinical presentation of rhinitis
runny nose, sneezing, itching, nasal congestion + obstruction
65
management of allergic rhinitis
glucocorticoids, antihistamines, muscarinic receptor antagonists, cysteinyl leukotriene receptor antagonists, vasocontrictors
66
causes of sinusitis
caused by sinuses being blocked (eg in the common cold) which allows bacteria to grow
67
clinical presentation of sinusitis
frontal headache, toothache, nasal discharge
68
stridor
inspiratory wheeze due to large airway obstruction
69
causes of stridor
in children- most commonly infections or a foreign body obstructing airway
adults- most commonly due to tumours
also anaphylaxis and trauma
70
acute bronchitis
infection of the bronchi, most cases are viral
71
pathophysiology of bronchitis
infection irritates and inflames bronchi -> increased mucus production -> productive cough
72
causes of bronchiolitis
mostly due to respiratory syncytial virus, common in young children, winter epidemics
73
clinical presentation of influenza
high fever, chills, headache, malaise, myalgia, cough, nasal congestion, fatigue
74
cause of whooping cough
acute trachea bronchitis caused by bordetella pertussis
75
community acquired organism causing pneumonia, most common
streptococcus pneumonia
76
organism causing pneumonia in the elderly and patients with COPD
haemophilus influenza
77
organism causing pneumonia in PWIDs, often following influenza
staph aureus
78
atypical causes of pnuemonia
legionella (contaminated water droplets), mycoplasma pneumonia (peaks every 4 years), coxiella burneti (farming), chlamydia psittaci (birds), klebsiella (from alcoholism), pneumocytis jiroveci (immunocompromised)
79
pathophysiology of lobar pneumonia
confluent consolidation involving a complete lung lobe, most commonly strep pneumoniae (community acquired) in otherwise healthy adults
80
pathophysiology of bronchopneumonia
infection staring in airways and spreading to adjacent alveolar lung, often seen in context of pre-existing disease
81
signs and symptoms of pneumonia
rigors, crackles + rub, tachypnoea, dyspnoea, pleuritic chest pain, cough/high fever (younger), confusion/diarrhoea/reduced mobility (older)
82
hospital investigations for pneumonia
FBC, CRP, U+E, CXR, sputum examination + culture, blood culture, legionella + pneumococcal urinary antigens
83
CURB65 risk score used to assess severity of pneumonia
Confusion
Urea > 7mmol
Resp rate > 30
BP- systolic < 90 or diastolic < 60
> 65 years
84
rust coloured sputum
S. pneumonia
85
green sputum
Pseudomonas, Haemophilus
86
red currant-jelly sputum
klebsiella
87
foul smelling + bad tasting sputum
anaerobes
88
management of community acquired pneumonia, CURB 0-2, CURB 3-5, ICU
0-2: amoxicillin IV/PO
3-5: co-amoxiclav + doxycycline
ICU: co-amoxiclav + clarithromycin
89
management of hospital acquired pneumonia, non-severe and severe
non-severe: amoxicillin PO + metronidazole
severe: amoxicillin IV + gentamicin + metronidazole
90
management of atypical pneumonias
doxycycline
except legionella- clarithromycin/erythromycin or levofloxin
91
92
intrapulmonary abscess
parenchymal necrosis with confined cavitation that results from a pulmonary infection
93
causes of intrapulmonary abscess
usually a preceding illness, eg pneumonia
can be due to a septic embolism, eg PWID
94
clinical presentation of intrapulmonary abscess
pneumonia that worsens despite treatment, weight loss, cough, lethargy
95
investigations for an intrapulmonary abscess
CXR- walled cavity
CT can be used to differentiate between an empyema and an abscess
96
management of intrapulmonary abscess
broad spectrum antibiotics, surgical drainage/resection may be necessary
97
causes of tuberculosis
mycobacterium tuberculosis is the main causative organism in humans (rod-shaped gram positive bacillus, acid fast), M. bovis can jump the species barrier from cattle to humans
98
pathophysiology of tuberculosis
type 4 hypersensitivity-
pathogen reaches alveoli
phagocytosed in alveoli and carried to hilar lymph nodes -> immune activation
granulomatous response in nodes with caseous necrosis in granulomas
infection cleared/becomes latent
99
miliary TB
infection is overwhelming and spreads throughout the body, setting up many foci of infection
100
clinical presentation of TB
cough +/- haemoptysis, dyspnoea, fever + chills, night sweats, fatigue, weight loss, erythema nodosum
101
CXR with active TB
shadows, lesions, consolidation
ghon focus in periphery of mid zone, bilateral hilar lymphadenopathy, miliary shadowing
102
zhiel-neelson stain
tests for acid fast bacteria, stain red against a blue background, test for TB
103
test for latent TB
tuberculin skin test
104
management of active TB
rifampicin, isoniazid, pyranzinamide and ethambutol for 4 months
105
management of latent TB
rifampicin and isoniazid for 3 months, isoniazid for 6 months
106
causes of lung cancer
inhalation of carcinogens- smoking, asbestos (crocidolite/amphibole is most dangerous type due to straight structure), pollution
107
pathophysiology of small cell lung cancer
25%, rapidly progressive disease, worst prognosis, central tumour, associated with ACT hormone secretion (Cushing's) and SIADH (hyponatremia)
108
types of non-small cell lung cancer
adenocarcinoma, squamous cell carcinoma, large cell carcinoma
109
adenocarcinoma- lung cancer
type most likely to be found in non-smokers, most closely linked with asbestos, peripheral tumour
110
squamous cell carcinoma- lung cancer
most common type in smokers, central tumour, local spread common, ectopic PTHrP release -> hypercalcaemia
111
large cell carcinoma- lung cancer
2nd worse survival rate, early metastases, peripheral tumour
112
paraneoplastic syndromes
lambert-eaton syndrome- disorder of neuromuscular transmission causing muscle weakness, thrombophlebitis- blood clot formation, anaemia, hypertrophic pulmonary osteoarthropathy- joint stiffness + pain in wrists and ankles
113
commons sites of metastases from a primary lung cancer
brain, liver, adrenal, bone
114
general symptoms of lung cancer
cough > 3 weeks, dyspnoea (due to airway obstruction), haemoptysis (due to erosion of blood vessel), chest/shoulder pain, weight loss, tiredness/lack of energy
115
pancoast's tumour
tumour in the lung apex infiltrates the brachial plexus causing Horner's syndrome (miosis, ptosis, anhidrosis)
116
tumour compressing laryngeal nerve causes
hoarse voice
117
tumour compressing pericardium causes
breathlessness, AF, pericardial effusion
118
tumour compressing oesophagus causes
dysphagia (difficulty swallowing)
119
tumour compressing SVC causes
puffy eyelids, headache, distension of the jugular veinds and veins on the chest (anastamoses)
120
signs of lung cancer
stridor, clubbing, enlarged liver, lymphadenopathy, tracheal deviation, reccurent pneumonia (tumours blocking bronchi stop mucosillary escalator from functioning)
121
investigations for lung cancer
CXR, biospy, bloods (low sodium, high calcium indicates malignancy)
122
CXR findings for peripheral and central lung tumours
peripheral- arise beyond the hilum, rarely visible on CXR
central- arise at/close to hilum, indicated by hilar enlargement and distal collapse/consolidation
123
peripheral lung cancers
adenocarcinoma and large cell carcinoma
124
central lung tumours
squamous cell carcinoma and small cell lung cancer
125
biospy for central and peripheral lung tumours
central- bronchoscopy and biopsy
peripheral- CT guided biopsy
aspiration of lymph nodes and pleural fluid
126
CT thorax, PET scan and USS uses in lung cancer
CT thorax- stages tumour
PET- detect metastases
USS- pleural effusion
127
management of SCLC
chemotherapy +/- radiotherapy
128
management of NSCLC
peripheral tumours with no metastases can be excised
chemotherapy +/- radiotherapy
129
mesothelioma
aggressive malignant tumour of the mesothelium, 90% arise from the pleura
130
cause of mesothelioma
most commonly asbestos, 20-40 years post exposure, most strongly associated with chrysotile asbestos fibres
131
clinical presentation of mesothelioma
pleural effusion (progressive dyspnoea, stony dull percussion), chest pain, weight loss
132
CXR in mesothelioma
pleural effusion, 'pleural mass with lobulated margin', may show pleural thickening
133
what will aspiration of pleural fluid in mesothelioma show
lymphocytes and low glucose
134
management of mesothelioma
only palliative (chemo/radio) as excision usually not possible, poor prognosis
135
management of a malignant pleural effusion
TALC (sclerosing agent), long term pleural catheter
136
pleural effusion
accumulation of fluid within the pleural space
137
causes of transudative pleural effusion
caused by disturbances in oncotic pressure, commonly cardiac failure and liver cirrhosis, low protein content (< 30g/L)
138
causes of exudative pleural effusion
fluids that have left the circulatory system and have gone into lesions or areas of inflammation, commonly due to malignancy or infection, high protein content (> 30g/L)
139
symptoms and signs of pleural effusion
chest pain, dry cough, dyspnoea, difficulty taking deep breaths, reduced chest expansion on affected side, stony dull percussion
140
straw coloured thoracentesis suggests
cardiac failure, hypoalbuminaemia
141
bloody thoracentesis suggests
trauma, malignancy, infection, infarction
142
turbid/milky thoracentesis suggests
empyema, chylothorax
143
foul smelling thoracentesis suggests
anaerobic empyema
144
food particles in thoracentesis suggests
oesophageal rupture
145
lymphocytes in pleural effusion sample suggest
malignancy or TB
146
neutrophils in pleural sample suggest
indicate an acute process
147
simple effusion vs complicated effusion
pH > 7.2 = simple effusion
pH < 7.2 = complicated effusion
148
amount of glucose in pleural effusion sample is low in
infection, TB, malignancy
149
treatment of simple effusion
treat with antibiotics only
150
treatment of complicated effusion
requires a chest drain and antibiotics
151
empyema
purulent fluid collection in the pleural space, most commonly caused by pneumonia
152
pathophysiology of empyema
simple pleural effusion (pH > 7.2, high glucose, neg gram stain) -> complicated pleural effusion (pH < 7.2, low glucose, pos gram stain) -> empyema (mostly aerobic organisms, sometimes anaerobes eg from severe pneumonia)
153
clinical presentation of empyema
slow to resolve pneumonia, typically patients partially recover then develop spike in temp
154
investigations for empyema
CXR- fluid level
USS- to confirm collection of pleural fluid
CT- differentiates between an empyema and an abscess
155
management of empyema
broad spectrum IV antibiotics (amoxicillin + metronidazole)
oral antibiotics directed towards bacteria once cultures come back (usually 5 weeks of co-amoxiclav)
chest tube drainage
156
chest tube drainage placement
5th ICS, midaxillary line in the safe triangle (anterior border of latissimus dorsi, posterior border of pectoralis major, axial line superior to nipple
157
causes of primary spontaneous pneumothorax
no underlying lung disease, usually result of rupture of a bulla
risk factors- tall thin men, smokers (esp cannabis)
158
cause of sencondary spontaneous pneumothorax
underlying lung disease
159
signs and symptoms of pneumothorax
acute onset pleuritic chest pain, dyspnoea, hypoxia, tachycardia, reduced breath sounds and chest expansion on affected side, hyper resonance on percussion
160
management of pneumothorax in an acutely unwell patient
aspiration (5th ICS, midaxillary line, safe triangle), if aspiration fails insert chest drain
161
pathophysiology of tension pneumothorax
one-way valve causes peogressively increasing pressure in the pleural space leading to mediastinal shift + cardiorespiratory compromise
162
clinical presentation of a tension pneumothorax
hypertensive, tachycardic, high resp rate, tracheal deviation, elevated JVP
163
management and emergency management of tension pneumothorax
needle aspiration until chest drain can be inserted
emergency- needle decompression (large gauge cannula inserted into 2nd/3rd ICS in midclavicular line)
164
causes of pulmonary venous hypertension
LV systolic dysfunction
mitral regurgitation/stenosis
cardiomyopathy eg alcohol, viral
165
primary arterial hypertension
primary disease of the pulmonary arteries/arterioles
166
secondary arterial hypertension
occurs due to underlying disease/known risk factors
heart disease (eg congenital shunts) and lung fibrosis (eg COPD, OSA, pulmonary fibrosis)
167
signs of pulmonary hypertension
dependent oedema, elevated JVP, RV heave at left parasternal edge, murmur of tricuspid regurgitation, loud P2, enlarged liver
168
investigations for pulmonary hypertension
ECHO doppler- estimates systolic pulmonary arterial pressure
right heart catheterization to confirm
ECG (right axis deviation, RBBB), CXR (cardiomegaly)
169
cor pulmonale
type of right sided heart failure, complication of pulmonary hypertension
increase RV afterload -> RV hypertrophy/dilation -> RSHF
170
oedema
accumulation of fluid in the interstitial spaces
171
causes of oedema
raised capillary pressure, reduced plasma osmotic pressure (malnutrition, hepatic failure), lymphatic insufficiency (lymph node damage), changes in capillary permeability (inflammation, histamine)
172
raised capillary pressure in LV failure causes which type of oedema
pulmonary oedema
173
raised capillary pressure in RV failure causes
peripheral oedema
174
pulmonary oedema
accumulation of fluid in the interstitial and intra-alveolar lung spaces, diffusion distance decreases so gas exchange compromised
175
peripheral oedema
oedema in tissues perfused by the peripheral vascular system, usually in the lower limbs
176
ARDS
non-cardiogenic pulmonary oedema and diffuse lung inflammation, typically secondary to an underlying illness
177
pulmonary causes of ARDS
chest sepsis, aspiration, inhalation injury, pulmonary contusion, transfusion-related lung injury
178
non pulmonary causes of ARDS
sepsis from a non-pulmonary source, acute pancreatitis, disseminated intravascular coagulation, drug overdose
179
pathophysiology of ARDS
injury (sepsis, severe trauma, pneumonia) -> inflammation (damages blood vessels) -> increased permeability of alveolar capillaries -> fluid accumulation in alveoli
180
symptoms of ARDS
acute onset resp failure that fails to improve with supplemental O2, severe dyspnoea, confusion, presyncope
181
signs of ARDS
elevated resp rate, bilateral lung crackles, low spO2
182
CXR finds in ARDS
bilateral alveolar infiltrates, without other features of HF (such as cardiomegaly, kerly B lines)
183
cause of neonatal RDS
premature infants are deficient in surfactant
increased effort in expanding the lungs damages cells, including blood vessels -> fluid leaks out into alveoli
184
restrictive lung disease
pulmonary disorders characterised by reduced lung volumes
185
causes of intrinsic restrictive lung diseases
diseases of the lung itself resulting in impaired alveolar gas exchange- type I oxygen failure
pneumonia, TB, intersititial lung diseases
186
causes of extrinsic restrictive diseases
thoracic/extra thoracic- obesity, ascites, diaphragmatic palsy, kyphoscoliosis
neuromuscular disorders- motor neuron disease
pleural diseases- diffuse pleural thickening, mesothelioma, large pleural effusions
impair alveolar ventilation- type II oxygen failure
187
symptoms of restrictive lung disease
progressive dyspnoea +/- dry cough, CO2 retention- headache, confusion, lethargy
188
signs of restrictive lung disease
finger clubbing, obese/kyphosis/scoliosis, fibrotic crepitations, cyanosis, CO2 retention- flushed skin, bounding pulse, flapping tremour
189
PFTs in restrictive lung disease
resitrictive- FVC and FEV1 both decreased, FEV1/FVC ratio normal
190
ABGs in restrictive lung disease
type I/II resp failure, increased bicarbonate indicates chronic hypercapnia, also decreased PaO2
191
type I respiratory failure
hypoxaemia (low O2) with normal or low CO2, primarily a problem of gas exchange
192
type II respiratory failure
reduced movement of air in and out of the lungs (hypoventilation), leading to hypercapnia and associated hypoxia
193
management of restrictive lung disease
supportive- oxygen, CPAP, NIV
194
hypersensitivity pneumonitis
acute or chronic hypersensitivity type III reaction to an antigen
195
causes of hypersensitivity pneumonitis
allergic reaction affecting small airways + alveoli in response to inhaled antigen
common antigens- thermophilic bacteria (farmers lung), avian proteins (bird fanciers lung), fungi (malt workers lung)
196
pathophysiology of hypersensitivity pneumonitis
inhaled antigens deposited in lung, stimulate antibody formation -> immune complex formation
complement activation -> inflammation
persistant inflammation leads to fibrosis
197
clinical presentation of acute hypersensitivity pneumonitis
symptom onset 4-6 hours following exposure
malaise, dry cough, pyrexia, dyspnoea
198
clinical presentation of chronic hypersensitivity pneumonitis
results from repeated low dose antigen exposure over time
may be crackles and clubbing
199
CXR findings in acute hypersensitivity pneumonitis
widespread pulmonary infiltrates
200
CXR, CT and PFT findings in chronic hypersensitivity pneumonitis
CXR- pulmonary fibrosis, most commonly in upper zones
CT- patchy micronodules of fibrosis in upper lobes
PFTs- restrictive
201
management of acute hypersensitivity pnuemonitis
oxygen, steroids, antigen avoidance
202
management of chronic hypersensitivity pneumonitis
antigen avoidance, oral steroids if dyspnoea/low gas transfer, antifibrotic therapy (pirfenidone, nintedanib)
203
idiopathic pulmonary fibrosis
progressive interstitial fibrosis of unknown cause
204
causes of idiopathic pulmonary fibrosis
most common in smokers and older people
205
pathophysiology of idiopathic pulmonary fibrosis
repetitive injury to alveolar epithelium, leads activation of repair pathways
wound healing mechanisms become uncontrolled -> fibrosis
terminally, lung structure replaced by dilated spaces surrounded by fibrous walls 'honeycomb'
206
signs and symptoms of idiopathic pulmonary fibrosis
progressive dyspnoea, dry cough, weight loss, fatigue, malaise, clubbing, cyanosis, bilateral fine inspiratory crackles
207
CXR and CT findings in idiopathic pulmonary fibrosis
bilateral lower zone reticulonodular shadows
208
management of idiopathic pulmonary fibrosis
antifibrotic drugs slow progression (nintedanib, pirfenidone), oxygen if hypoxic, lung transplant in young patients
209
pneumoconiosis
lung disease caused by mineral dust exposure
210
causes of pneuoconiosis
heavy prolonged exposure to asbestos -> asbestosis
15-20 years coal dust exposure -> coal workers pneumoconiosis
15-20 years exposure to silica -> silicosis
211
caplans syndrome
caused by occupational dust inhalation in patients with rheumatoid arthritis
212
pathophysiology of pneumoconiosis
inhaled particles not cleared by mucociliary clearance become encased in macrophages + set off inflammatory responses -> fibroblast proliferation + collagen deposition
213
clinical presentation of simple pneumoconiosis
usually asymptomatic, can progress to development of progressive massive fibrosis
214
clinical presentation of progressive massive fibrosis (complicated pneumoconiosis)
dry cough, progressive dyspnoea, clubbing, inspiratory crackles, no pain- if asbestos + pain think malignancy
215
CXR findings in simple pneumoconiosis
non-calcified multiple round opacities in upper zone
216
CXR findings in complicated pneumoconiosis
bilateral, upper-mid zone fibrotic masses, develops from periphery to hilum
217
management of progressive massive fibrosis
bronchodilator, oxygen, pulmonary rehab
218
sarcoidosis
multisystem granulomatous (type IV) disorder to an unknown antigen
219
pathophysiology of sarcoidosis
inhalation of unknown antigen stimulates alveolar macrophages, T + B cells
failure to clear antigen -> persistent stimulation and granuloma formation -> tissue damage + fibrosis
220
clinical presentation of sarcoidosis
bilateral hilar lymphadenopathy, pulmonary infiltration, skin/eye lesions, crackles on lung auscultation
221
transbronchial biospy in sarcoidosis will show
non-caseating granulomata
222
management of chronic sarcoidosis
oral steroids, may need immunosuppression (methotrexate, azathioprine)
223
anti-glomerular basement membrane disease- goodpasture's syndrome
autoimmune disease, the co-existence of acute glomerulonephritis and pulmonary alveolar haemorrhage, more common in men
224
pathophysiology of goodpastures syndrome
type II antigen-antibody reaction leading to diffuse pulmonary haemorrhage and glomerulonephritis
225
clinical presentation of goodpastures syndrome
presents as acute kidney injury caused by rapidly progressive glomerulonephritis, accompanied by pulmonary haemorrhage
226
symptoms of goodpastures syndrome
chills, fever, nausea, weight loss, chest pain, anaemia, haematuria, arthralgia
227
urinalysis findings in goodpastures syndrome
low grade albuminuria, gross/microscopic haematuria, RBC casts
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diagnosis of goodpastures syndrome
anti-GBM antibodies are diagnostic
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management of goodpastures syndrome
plasmapheresis to remove circulating antibodies, immunosuppression (prednisolone and cyclosphamide) to prevent further antibody production
