Respiratory - Breathlessness: Restrictive Lung Disease Flashcards

(374 cards)

1
Q

Primary restrictive lung disease

A

Disease of lung parenchyma

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2
Q

Secondary restrictive lung disease

A

Chest wall and pleural disease

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3
Q

What does restrictive lung disease cause difficulty in

A

Inhaling

Reduced elasticity resulting in poor lung expansion

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4
Q

What are some mediators of infl

A

Leukocytes
Cytokine
Complement pathway proteins

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5
Q

The 5 R’s of infl & repair

A
Recognition of the injurious agent 
Recruitment of the leukocytes 
Removal of the agents 
Regulation of the response 
Resolution/ repair
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6
Q

Why does lung fibrosis result in SOB

A

Pts will have dyspnoea, tachypnoea, fine end inspiratory crackles

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7
Q

A/c lung injuries

A

ARDS

Pneumonia

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8
Q

Mortality of ARDS

A

50%

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9
Q

ARDS

A

A/c Resp Distress Syndrome

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10
Q

What is ARDS

A

Primary resp failure characterised by refractory hypoxaemia and bilateral radiological evidence of alveolar collapse
No other attributable cause

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11
Q

What does ARDS end in

A

Vascular non-responsiveness

Leading to multiorgan failure due ton c/c hypoxia and effects of infl mediators

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12
Q

How can causes of ARDS be classified

A

Direct/ indirect lung injury

Common and uncommon

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13
Q

Common causes of ARDS - direct lung injury

A

Pneumonia

Aspiration of gastric contents

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14
Q

Uncommon causes of ARDS - direct lung injury

A

Pulmonary contusion
Inhalation injury
Fat emboli
Reperfusion injury

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15
Q

Common causes of ARDS - indirect lung injury

A

Sepsis

Severe trauma and shock

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16
Q

Uncommon causes of ARDS - indirect lung injury

A

Post-cardiac surgery
Pancreatitis
Drug overdose

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17
Q

Phases of ARDS

A

Exudative
Regernative
Repair

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18
Q

Exudative phase of ARDS

A

Necrosis of Type 1 pneumocytes then exposure and subsequent loss of the underlying basement membrane, fibrin release +/- haemorrhage, pulmonary collapse

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19
Q

How long does the exudative phase of ARDS last

A

~7/7

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20
Q

Regenerative phase of ARDS

A

Proliferation of pneumocytes and connective tissue

Infl

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21
Q

How long does the regenerative phase of ARDS last

A

1-2 weeks post injury

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22
Q

Repair stage of of ARDS

A

Infl, proliferating fibroblasts in the connective tissue produce granulation tissue (organising pneumonia) and/ or fibrous scar tissue

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23
Q

How long does the repair stage of ARDS last

A

2 weeks plus

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24
Q

Why is long intensive care required in ARDS

A

Pt have v high mortality weeks after initial event

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25
Stages of pneumonia
A/c Early resolving Organising From here there'll either be complete resolution to normal or may go onto develop interstitial fibrosis
26
A/c stage of pneumonia
Neutrophil infiltrates into bronchi and alveoli
27
Early resolving stage of pneumonia
Fibrin, some neutrophils also macrophages, lymphocytes in air spaces
28
What can granulomatous responses in the lungs be caused by
Foreign bodies: inhaled exogenous substances e.g. beryllium Infections: TB, fungi Immune: hypersensitivity pneumonitis unknown sarcoidosis
29
What is hypersensitivity pneumonitis
A c/c disease of the lungs resulting from inhalation of foreign substances showing both granulomatous and hypersensitivity reactions Pathogen acts as a foreign antigen and triggers a local hypersensitivity reactions
30
What is hypersensitivity pneumonitis also known as
Extrinsic allergic alveolitis
31
Sources of exposure leading to hypersensitivity pneumonitis
``` Mouldy hay - Farmer's lung Mouldy grain - Grain handler's lung Pigeons, parakeets, fowl - Bird breeder's lungs Cheese mould - cheese worker's lung Paprika dust - Paprika splitter's lung Infested wheat - wheat weevil ```
32
C/c fibrosis ILD
``` Pnuemoconiosis Idiopathic pulmonary fibrosis/ UIP CTD Radiation Drugs ``` COP NSIP
33
COP
Cryptogenic organising pneumonia
34
NSIP
Non-spp interstitial pneumonia
35
Pneumoconiosis
'Dusty lung disease' Permanent alteration of lung structures due to the inhalation of mineral dust and the tissue recitations of the lung to its presence
36
Commonest pneumoconiosis
Silicosis
37
Where does pneumoconiosis tend to be worse
In the upper lobes | This is due to more aeration of the lower lobes and better removal of particulate material
38
Pulmonary reactions seen in pneumoconiosis
``` Macrophage accumulation w/ a little reticulin deposition Nodular or massive fibrosis Diffuse fibrosis Epitheliod and giant cell granulomas Alveolar lipoproteinosis Small-airway disease ```
39
Dust identification - pneumoconiosis
Clinical hx Radiology - distribution of lung changes, upper, lower zones, nodule and fibrosis Colour of the dust particles Histology - refractory particles, crystal shape
40
What are asbestos related diseases
A family of pro-infl crystalline hydrated silicates Crystals once phagocytosed activate an infl response which stimulates the release of proinfl factors and fibrogenic mediators Macrophages attempt to phagocytose and digest fibres
41
What are asbestos related diseases associated with
Number of pulmonary and extra pulmonary diseases
42
Sequela of asbestos related diseases
Pleural plaques Pleural effusions Pulmonary fibrosis (asbestosis) Malignancy - lung carcinoma, mesothelioma, extra pulmonary
43
Clinical findings of asbestosis
SOB Dry cough Finger clubbing
44
Macroscopic findings of asbestosis
Subpleural fibrosis particularly lower lobes
45
Microscopic findings of asbestosis
Intersititial fibrosis: peri bronchial and sub pleural ad the presence of ferruginous bodies
46
Resolution of asbestosis
The persistent c/c infl nature o d the disease is such that there is no disease resolution
47
What is idiopathic pulmonary fibrosis also known by
Its histological pattern: UIP
48
What is idiopathic pulmonary fibrosis
Progressive interstitial pulmonary fibrosis and reps failure caused by persistent epithelial injury and abnormal immune reaction
49
Cause of idiopathic pulmonary fibrosis
No known cause
50
Who do we see idiopathic pulmonary fibrosis in
Arises in genetically predisposed individuals who are prone to aberrant repair of recurrent alveolar injury
51
Secondary causes of lung fibrosis - Reactions to treatment
Drugs - various meds may cause a/c/ and c/c alteration in lung structure Radiation pneumonitis
52
Types of radiation pneumonitis
A/c | C/c
53
A/c radiation pneumonitis
1/12 - 6/12 after treatment | May resolve completely w/ steroids or go on to a hypersensitivity pnuemoniti pattern
54
C/c radiation pneumonitis
Results in pulmonary fibrosis | Can also occur without a/c phase
55
Secondary causes of lung fibrosis - lung in systemic disease
Pulmonary - CTD | Extra-pulmonary - condns that limit MSK system or pleura e.g. scoliosis, and soon, neurodegenerative disease
56
Residual and functional volumes in obstructive disease
Increased
57
Inspiratory residual volume and inspiratory capacity in restrictive disease
Decreases
58
Examples of restrictive lung diseases
Fibrotic changes Scoliosis Muscular dystrophy Ank spon
59
Atelectasis
Imperfect expansion off lungs Specifically of failure of lungs to expand fully at birth Congenital airway obstruction due to lack of surfactant in prematurity
60
Secondary atelectasis
Pulmonary collapse Pressure changes and alveolar gas not replenished (absorption collapse) - obstruction prevents free entry of air into lungs
61
Epidemiology of sarcoidosis
2nd most common c/c reps disease in <40s | Bimodal distribution - 25-40 and 65+
62
Aetiology of sarcoidosis
Poorly understood - related to genetic predisposition plus exposure to antigen Non-caeseating granulomas lead to organ damage and dysfunction
63
Which type of sarcoidosis manifestation has a better prognosis
A/c
64
What % of sarcoidosis develop c/c symptoms
1/3 | Usually have non-spp, systemic symptoms
65
Pathophysiology of sarcoidosis
Antigen and host protein form a poorly soluble compound --. APCs activate macrophages & dendritic cells, producing cytokines --> Th1 help form epithelium granulomas
66
What does sarcoidosis chronicity depend on
Ability to clear antigen
67
Genetic causes of sarcoidosis
Multiple area of the HLA region BTNL2 | ANXA11
68
Causes of sarcoidosis - exposures
Beryllium, silic, other dust Mycobacterium tuberculosis and Propionibactera catalases Moulds
69
How is sarcoidosis usually discovered
Incidental finding - no symptoms suggestive of sarcoidosis | Good prognosis
70
A/c presentation of sarcoidosis
Flu-like illnesses Lofgren's syndrome Good prognosis - >605 in 2 yrs
71
Lofgren's syndrome
Bilateral hilar lymphadenopathy Erythema nodusum Arthralgia
72
Who do we typically see Lofgren;s syndrome in
Northern Europeans/ caucasians
73
C/c presentation of sarcoidosis
``` Progressive symptoms (e.g. dyspnoea, cough) Associated extra-pulmonary disease and organ dysfunction ```
74
Eye involvement in sarcoidosis
``` Uveitis Lacrimal gland involvement Optic nerve involvement Glaucoma Granulomatous tissue within orbit ```
75
Neurological involvement in sarcoidosis
Neuropathy Meningitis Raised ICP Psychiatric problems
76
Resp tract involvement in sarcoidosis
Lymphadenopathy ILD/ scarring Airway obstruction and stenosis
77
Skin involvement in sarcoidosis
Erythema nodusum S/c nodules Lupus pernio
78
MSK involvement of sarcoidosis
Myositis Arthralgia and arthritis Entheistis
79
Cardiac involvement in sarcoidosis
Arrhythmia Ventricular infiltration and failure SCD
80
GI and GU involvement in sarcoidosis
Nephrocalcinosis IBD mimic Infertility Obtruscive liver disease
81
What metabolic disturbance is seen in sarcoidosis
Hypercalcaemia
82
Eye symptoms in sarcoidosis
Eye pian Visual disturbance Vision loss
83
Resp symptoms seen in sarcoidosis
Breathlessness (exertional) Cough Wheeze
84
Neuro symtpoms seen in sarcoidosis
Seizures Headaches Cranial nerve palsies
85
GI and GU symptoms seen in sarcoidosis
Abdominal pain Haematuria Deranged LFTs Change in bowel habits
86
Lymphadenopathy seen in pulmonary sarcoidosis
Mediastinal and hilar | bIlateral and symmetrical
87
Parenchymal disease seen in pulmonary sarcoidosis
Nodularity following bronchovacsular bindles and adjacent to tissues Nodular disease (incl conglomerate masses) Fibrosis
88
Airway disease in pulmonary sarcoidosis
Asthma-like symptoms/ bronchial hyper-reactivity (cough predominant) Airway stenosis and occlusion
89
Which cardiac manifestations of sarcoidosis require mx
``` Dyspnoea Collapse CM SCD/ arrythmias Pulmonary HTN ```
90
Which neurological manifestations of sarcoidosis require mx
Seizures Aseptic meningitis Cranial nerve palsies
91
Which metabolic manifestations of sarcoidosis require mx
Hypercalcaemia | Hypercalciura/ renal calculi
92
Which opthalmic manifestations of sarcoidosis require mx
Uveitis | Visual loss
93
Sarcoidosis mimics
C/c berylliosis - clinically identical but w/ clear exposure to beryllium Granulomatous reactions to malignancy - context of known malignancy Granulomatous - lymphocytic ILD (occurs in immune deficiency) Drug reactions - anti-TNF monoclonal antibodies
94
Ix for sarcoidosis
Bloods PFTs CXR preceding tio CT chest ECG (look for heart block)
95
Bloods for sarcoidosis
FBC - lymphopenia, anaemia U&Es - renal dysfunction LFTs - derangement (obstructive or hepatotoxic) Serum ACE - elevated commonly but NOT spp Ig - association w/ Ig defences esp IgA
96
Pulmonary function tests in sarcoidosis
Any pattern can occur (obstructive most common)
97
Radiology for sarcoidosis
Looking for subtle parenchymal changes in lungs or any asymmetry to suggest alternative pathologies
98
CXR staging for sarcoidosis
Stage I to IV
99
Stage I sarcoidosis - radiological
Bilateral hilar lymphadenopathy
100
Stage I sarcoidosis - radiological
Bilateral hilar lymphadenopathy | Increased paratracheal stripe
101
Stage II sarcoidosis - radiological
BHL and pulmonary infiltrates
102
Stage III sarcoidosis - radiological
Pulmonary infiltrates without BHL
103
Stage IV sarcoidosis - radiological
Advanced pulmonary fibrosis Loss of lung volume Apical fibrotic change
104
Which stage of sarcoidosis is most pts in
Stage I | Reolustion of 60-90%
104
Which stage of sarcoidosis is most pts in
Stage I | Reolustion of 60-90%
104
Which stage of sarcoidosis is most pts in
Stage I | Resolution of 60-90%
105
CT findings for sarcoidosis
Hilar and mediastinal nodes - symmetrical, bilayer enlargement Nodules Fibrotic changes
106
Bronchoscopy for pulmonary sarcoidosis
Pts are likely to require biopsy from lungs | Done at same time
107
Possible targets for lung biopsies - pulmonary sarcoidosis
Endobronchial lesions – cobble stoning Transbronchial biopsies – more risky EBUS-TBNA – biopsy of lymph nodes, helps rule out metastaic malignancy
108
Treatment for pulmonary sarcoidosis
Majority of pts will not require immediate treatment; raise w/ more severe disease require early mx
109
Reasons to initiate treatment in sarcoidosis
Wells Law Danger of damage of organs (incl preventing mortality) Improve QoL
110
Reasons to initiate treatment in sarcoidosis
Wells Law Danger of damage of organs (incl preventing mortality) Improve QoL
111
1st line drug of sarcoidosis
40-60mg Prednisolone - weaned over 6-18 months
112
1st line drug of sarcoidosis
40-60mg Prednisolone - weaned over 6-18 months
113
2nd line drugs for sarcoidosis
MTX - 10-15mg once weekly | Azathioprine - 1-3mg/kg - good for neuro-sarcoid
114
SE of MTX
Cytopenias GI side-effects Pneumonitis
115
SE of azathioprine
Cytopenias GI side effects LFT derangement
116
3rd line drugs for sarcoidosis
Mycophenolate Mofetil Hydroxychloroquine Anti-TNF
117
Mycophenolate
Cytopenias GI side effects LFTs derangement
118
SE of hydroxychloroquine
Optic neuritis
119
Follow up for Lofgren's syndrome (Stage I CXR)
6 monthly for 2 yrs
120
Follow up for Stage II - IV sarcoidosis CXR
3-6 monthly or annually Depending on clinical suspicion of a change in disease behaviour No discharge
121
Follow up of pulmonary sarcoidosis pts who've been withdrawed from steroid therapy
2-3 monthly or 3-6 monthly Minimum 3 years after cessation No discharge
122
Mortality and sarcoidosis
Mortality attributable to sarcoidosis in pts w/ significant disease manifestations are more like 25%
123
Diffuse Parenchymal Lung diseases (DPLD)
Vast majority cause restrictive PFTs Gradual onset breathlessness Frequently cause cough Will hear inspiratory carpitations
124
Causes of DPLD
Idiopathic interstitial pneumonia Spp cause - drugs, hypersensitivity pneumonitis, CTD Granulomatous - sarcoidosis
125
What is seen in the interstitium
ECM | Fibroblasts
126
Aetiology of DPLD
Drugs, therapies, iatrogenic - bleomycin, amiodarone Inhalation of dusts (occupation, environment) CTD (collagen diseases) Unknown (idiopathic)
127
Idiopathic Interstitial Pneumonia
``` IDO NSIP COP Resp bronchiolitis ILD (R-BILD) Desquamative Interstitial Pneumonia (DIP) A/c interstitial pneumonia ```
128
CT of UIP
Honeycombing +/- traction bronchiectasis
129
Epidemiology of NSIP
40-50yrs M>F Better prognosis than IPF May be associated w/ CTD
130
CT of NSIP
Ground glass | Reticular shadowing
131
CT of COP
Consolidation or nodules
132
R BILD
Exaggerated bronchiolitis response to smoking
133
CT of R BILD
Patchy ground glass Centrilolobular micro nodules Regional attenuation
134
Who do we see DIP in
Heavy smokers
135
CT of DIP
Ground glass opacities | Bronchial thickening
136
CT of a/c interstitial pneumonia
Consolidation | Ground glass
137
What type of hypersensitivity reaction causes hypersensitivity pneumonitis
Type III or Type IV
138
Mx of hypersensitivity pneumonitis
Removal from antigen | Steroids
139
Examination finding in pts w/ restrictive lung disease
Fibrosis - fine end inspiratory crackles/ crepitation Sarcoidosis - normal Hypersensitivity pneumonitis - crackles, wheeze, squeaks
140
Main ix for restrictive lung disease
Pulmonary function tests Bloods Radiology
141
Blood for restrictive lung disease
FBC (eos) U&ES LFT Serological - ANA,, RhF, anti-CCP, spp IgG's
142
Other tests for restrictive lung disease
ECG Echo Radiology - CXR
143
HRCT of chest in restrictive lung disease
``` Ground glass - non spp Consolidation Reticular shadowing* Traction bronchiectasis* Honeycombing* ``` * signs of fibrosis
144
Ground glass appearance on HRCT
Hazy Opoacity that doesn't obscure the associated pulmonary vessels
145
What causes a ground glass appearance
Parenchymal abnormalities - infl or fibrosis
146
MDT for restrictive lung disease
Physician Radiologist Pathologist
147
Treating fibrosis in restrictive lung disease
Anti-fibrosis | Only for IPF (progressive fibrosis on named pt basis)
148
Treatment for infl in restrictive lung disease
Corticosteroids MTX/ hydroxychloroquine Azathioprine/ mycophenolate Cyclophosphamide
149
Non Pharma treatment for restrictive lung disease
``` Education Pt support group Pulmonary rehab Oxygen therapy Palliative care - early referral provides better outcomes ```
150
Pathophyisology of idiopathic pulmonary fibrosis
Epithelial-Fibroblast Pathway (fibroblastic foci) ---> interstitial infl ---> fibrosis (collagen)
151
Main diagnostic tool for restrictive lung disease
HRCT
152
Prognosis of restrictive lung disease
No biomarkers can predict prognosis, only clinical | Scoring calculators - GAP score
153
Mx for restrictive lung disease
``` Oxygen Lung transplant Pirfenidone Nintedanib Pulmonary rehab ```
154
When would anti-fibrotics be given
When FVC 50-80% predicted
155
Anti-fibrotics given for restrictive lung disease
Pirfenidone | Nintedanib
156
Pirfenidone action
Reduced fibroblast proliferation | Inhibits production of TGF-beta production and collagen stimulation
157
SE of pirfenidone
Nausea | Photosensitivity rash
158
Nintedanib
Triple growth-factor receptor inhibitor | Platelet derived, fibroblast and vascular endothelial growth factor receptor (PDGF, FGF, VEGF)
159
SE of Nintedanib
Diarrhoea
160
Symptoms seen in a/c exposure causing HS pneumonitis
Fever SOB Cough
161
What is seen in c/c exposure causing HS pneumonitis
Progressive fibrosis
162
Type 1 resp fialure
Hypoxia W/OUT hypercapnia
163
Type 2 resp failure
Hypoxia and hypercapnia | Can be a sign of lfe-threatening asthma attack
164
Common occupational lung diseases
Asbestos related diseases Asthma (occupational) Pneumoconiosis Beryllium disease
165
Susceptible trades and industries for occupational lung diseases
``` Construction Engineering Welding Foundries/ quarries and potteries Motor vehicle repair Stonemasons Farmers ```
166
When were asbestos fully banned
1999
167
Asbestos
Cheap, strong, fire and heat resistant fibres used in building
168
Why are asbestos dangerous
Fibres <3 microns diameter ca reach alveoli
169
When do symptoms of mesothelioma appear
Until 20 to 50+ years after exposure
170
Manifestations of asbestos exposure
``` Pleural plaques Diffuse pleural thickening Malignant mesothelioma Asbestosis Lung cancer Laryngeal cancer ```
171
Inhalation of asbestos fibres
Particles <10um may penetrate alveoli Long straight fibres can reach alveoli despite length \ Longer fibres more resistant to clearance by phagocytosis
172
What is asbestosis
Form of pneumoconiosis caused by inhalation of asbestos fibres which is characterised by scarring and infl of lung tissue
173
Is asbestosis reversible
No A c/c and irreversible condn which symptoms develop several decades following exposure Symptoms may seriously progress --> affecting ADL --> fatal complications
174
Clinical features seen in asbestosis
``` Dyspnoea Non-productive cough Wt loss Fine end inspiratory crackles Finger clubbing Susceptibility & deterioration associated w/ smoking ```
175
Ix for asbestosis
``` Spirometry CXR HRCT Lung biopsy Transfer factor ```
176
Spirometry in asbestosis
Usually restrictive
177
Transfer factor seen in asbestosis
Reduced
178
CXR in asbestosis
Fine nodular shadoiwng
179
HRCT for asbestosis
More sensitive than CXR at showing fibrosis
180
Lung biopsy for asbestosis
Interstitial fibrosis and asbestos bodies
181
Asbestos exposure mx
Supportive | Stop smoking
182
Malignant mesothelioma
Form of cancer that principally affects the pleura and peritoneum Strong association w/ exposure to asbestos
183
Why are most cases of malignant mesothelioma diagnosed at an advanced stage
As symptoms are non-spp and appear late
184
Symptoms of malignant mesothelioma
Chest pain SOB Pleural effusion
185
Industrial injuries disablement benefit
``` Asbestosis Mesothelioma Primary carcinoma lung Diffuse pleural thickening Non pleural plaques ```
186
How can work aggravate asthma
Provokes symptoms of pre-existing asthma | A/c transient airway narrow after exposure to resp irritant such as dust, smoke, DO2, cold, exercise
187
Irritant induced occupational asthma
Single exposure to high level of irritant e.g. NH3, Cl2 (RADS) C/c moderate level exposure - more delayed onset of symptoms
188
RADS
Respective Airways Dysfunction Syndrome
189
Sensitiser induced occupational asthma
Asthma caused by immunological sensitisation to agents in the agents in the workplace (Type 1 HS) Latency between weeks and yrs
190
What % of adult asthma is occupational
10%
191
Is occupational asthma a 'prescribed diseases'
Yes | Pt may be eligible to industrial injuries disablement benefits
192
Initial px of occupational asthma
Initially symptoms of wheeze, dyspnoea and chest tightness at work or after work, improving over weekends & holidays Over time this pattern can be lost
193
Occupations that might cause asthma
``` Paint spraying Cleaners Bakers Lab workers Carpentry ```
194
Dx of occupational asthma
``` Hx Serial peak flow looking for >20% variation across shift and Lowe/ more variable peak flow on workdays RAST (spp IgE), skin prick Spp inhalation challenge Workplace challenge ```
195
Mx of occupational asthma
Treatment as per BTS guideline | Symptoms usually resolve if exposure in eliminated
196
Hierarchy of control measures for occupational asthma
Elimination or substitution Engineering controls e.g. enclose work process Admin controls e.g. relocation to a diff job/ work area PPE
197
What % of people w/ occupational asthma remain in same job
25% | 25% remain w/ same employer in an alternative job
198
Long term changes caused by localised infl response in pneumoconiosis
Fibrosis Necrosis Cavitation Coalescence into larger masses
199
Mechanisms protecting lungs from inhaled particles
Particles >20um trapped in nasal cavity 10-20um trapped in upper branches of resp tract, cleared by mucocilliary escalator Particles may also be transported to lymphatics The mechanisms can be overwhelmed by large amounts of particulate matter
200
Coal Workers Pneumoconiosis
Tissue reaction to dust in the lung parenchyma Often asymptomatic Usually (but not always) non-progressive if removed from exposure
201
IX for simple CWP
Spiro - obstructive (emphysemna) or restrictive (fibrosis) | CXR - nodular opacities
202
PMF (progressive massive fibrosis)
Fibrotic masses in upper or middle zones, 3-10cm diameter, can cavitate Lung function loss mainly obstructive
203
Symptoms seen in PMH
``` Dyspnoea Productive cough (black sputum) ```
204
A/c silicosis
Heavy exposure to dust respirable crystalline silica (silicon dioxide) Direct cytotoxicity causing alveolitis Progressive & often fatal over few months
205
Symptoms in a/c silicosis
Progressive dyspnoea & cough
206
CXR for a/c silicosis
Pulmonary oedema
207
Mx of silicosis
No spp treatment
208
Nodular/ c/c silicosis
10-15 yrs of lower level exposure Cough & dyspnoea May see silico-TB
209
Sources of exposure of silicon dioxide
Mining Constrcution Stone working Abrasive blasting
210
Silicosis CWP
Opacities larger than in CWP More marked in upper lobes May see pleural thickening 'Egg shell" calcification hilar lymph nodes
211
When does COPD become a prescribed disease
If coal miner > 20 yrs
212
Occupational resp infections
Viral resp tract infections TB Legionnaires Psittacosis (Chlamydia psittaci)
213
IPF lungs
Irreversibly enlarged, damaged bronchioles and distorted alveoli Honeycombing - clustered cystic air spaces Fibrosis between alveoli greatly decreases gas exchange
214
UIP fibrosis on HRCT
``` Sub -pleural Basal predominance Reticular shawoing Honeycombing Traction bronchiectasis ```
215
MDT team for ILD
``` ILD consultant ILD CNS Radiologist Thoracies Pathologist ```
216
Pulmonary involvements in CTD
Pleural diseases - thickening, effusion Airway complications ILD Pulmonary Vascular disease - pulmonary HTN Opportunistic infections due to immunosuppression Drug toxicity - MTX
217
Median survival of IPF/ UIP
2.5 yrs - 3.5 yrs
218
How long is steroid. treatment required for COP
6/12
219
AIP
A/c interstitial pneumonia Rapid onset of symptoms, dramatic clinical deterioration Hypoxaemia, resp failure
220
CTD associated w/ ILD
``` RhA SScl SLE Dermatomyositis Sjorgen's syndorme Mixed CTD ```
221
Epidemiology of IILD - RhA
More common in males
222
Prognosis for ILD - RhA
Prognosis varies depending on subtype of ILD and degree of fibrosis Progressive symptoms of breathlessness is a strong predictor of poor prognosis
223
Ix for ILD - RhA
Lung volume of CXR Extent of fibrosis on HRCT Serial FVC on lung function
224
Relation between RhA symptoms and ILD
No relation between control of joint symptoms and course of ILD Progressive ILD can precedent synovitis or occur before onset of joint disease
225
ILD - RhA mx
Anti-TNF alpha | Corticosteroids - good response in pts w/ COP subtype
226
Which subtype of SScl is more frequently associated w/ ILD
DcSScl | Patients w/ dcSSc and lung involvement have 5-year survival < 50%
227
Relation between SScl symptoms and ILD
Extent of skin evolvement doesn't correlate w/ severity of lung disease Resp symtoms rarely preced Majority of pts have progressive symptoms
228
Mx of ILD - SScl
Directed at detecting ILD early Various immunosuppressive agents e.g. steroids, colchicine, D-pencillamine Cyclophosphamide is most effective
229
Manifestations of ILD in SLE
Usually a.c - a/c lupus pneumonitis w/ alveolar haemorrhage is well known Repeated episodes may cause residual and c/c fibrosis
230
Mx of lupus pneumonitis
Heavy immunosuppression and plasmapheresis - corticosteroids and azathioprine/ cyclophosphamide Mortality as high as 50%
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Relation between Dermatomyositis symptoms and ILD
ILD preceded skin or muscle manifestation in about 1/3 of pts
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How may ILD in dermatomyositis present
Rapidly progressive AIP Slowly progressiveing symptoms (UIP) or COP Abnormal imaging/ PFT but no resp symptoms
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Why might dermatomyositis pts have SOB
Due to ILD or 2' to pulm HTN, cardiac involvement or muscle weakness
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When are corticosteroids useful in ILD- DM
Those w/ a/c onset illness
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Poor prognostic features of ILD-DM
Older age group Short hx Dysphagia Inadequate response to treatment
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Subtypes of ILD seen in Sjogrens
UIP/ IPF – worst prognosis NISP COP
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Resp symtoms seen in Sjorgrens
Cough Breathlessness Will hear fine ends inspiratory crackles
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Usual mx for ILD -sjorgrens
Corticosteroids and azathioprine & cyclophosphamide
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ILD in MCTD
ILD spectrum similar to that seen in SScl | Degree of ;lung forbrosis more severe in pts w/ SScl features
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Common symptoms in MTX lung injury
Cough Dyspnoea Fever
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Pulmonary function tests in MTX lung injury
Restrictive defects | Low diffusion capacity
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CXR for MTX lung injury
Alveolar infiltrates Reticonodular shadowing Predominantly diffuse or lower lobe involvement
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HRCT for MTX lung injury
Patchy ground glass shadowing
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Mx of MTX lung injury
Discontinuation of therapy or corticosteroids (for breathlessness) Majority recover well and 25% experience reoccurrence once MTX in reintroduced (worse on high doses)
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Which pts have the lowest incidence of MTX lung injury
Pts on low dose MTX
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Anti-TNF treatment and ILD
Fatal exacerbations in pts w/ pre-existing ILD w. infliximab
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Assessment of iLDN in CTD pts
High clinical suspicion w/ good hx taking Examination - fine end inspiatry crackles CXR, PFT and HRCT Biopsy
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If there's rapid deterioration in pts on immunosupression, what should come come to mind
Pneumocystis jiroveci pneumonia
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What approach should be taken to mx CTD pts w/ ILD
MDT
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Where does the nasal cavity extend from
Anterior nares to nasopharynx
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unction of nasal cavity
Warm, filer and humidify air
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Medial wall of nasal cavity
Septal cartilage Perendicular plates of ethmoid & vomer Some palatine, maxillary and sphenoid
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Floor of nasal cavity
Roof of oral cavity Palatine process of maxilla Horizontal part palatine bones
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Roof of nasal cavity
Nasal cartilages | Nasal and frontal bones
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Lateral walls os nasal cavity
3 turbinates (conchae)
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Space below each turbinate
Meatus
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Space above each turbinate
Sphene-ethmoidal recess
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Paranasla sinuses
Group of air filled spaces that lie in bones of skull and drain into nasal cavity
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Paired air sinuses
Frontal Maxillary Sphenoid Ethmoid
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Floor of oral cavity
Tongue | Sub-lingual gland
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Roof of oral cavity
Palate (hard, soft, uvula)
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Dentition in oral cavity
Adult - permanent 32 | Child- deciduous 20
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Components of oral cavity
``` Floor Roof Dentition Cheeks and lips Tonsils ```
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Where is the pharynx found
Base of skull to C6
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How many paired constrictors does the pharynx have
3 - superior, middle and inferior
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What does the lining of the pharynx reflect
Where it is | Rest epithelium in nasopharynx and stratified squamous epithelium in oro- and laryngopharynx
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Innervation of pharynx
Pharyngeal plexus - vagus and glossopharyngeal nerves
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Whatcare the skeletal elements of the larynx joined bu
Thyrohyoid membran Cricothyroid membrane Aryepiglottic membrane
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Where is the vocal cord found
Fold between thyroid and arytenoid
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What is the space between false and true cords called
Sinus (ventricle of larynx)
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Mucosal innervation of larynx
Above vocal cord - internal laryngeal | Cord level and below - recurrent laryngeal nerve
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Extrinsic muscles of the larynx and hyoid bone
Suprahyoid and stylopharyngess - elevate hyoid and larynx as a whole Infra hyoid - depress the hyoid and larynx as a whole
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Intrinsic muscles of the larynx
``` Cricothyroid Tyro-arytenoid Posterior cricoid-arytenoid Lateral rico-arytenoid Transverse and oblique arytenoids Vocalise ```
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Action of the cricothyroid
Covers anterior cricoid superiorly and inferiorly stretches and covers vocal ligaments
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Action of thyro-arytenoid
Relaxes vocal ligament
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Action of posterior crico-arytenoid
Adducts vocal fold | As well as lateral crico-arytenoid
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Action of transverse and oblique arytenoids
Adduct arytenoids closing posterior rim of glottis
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Action of vocalist
Relaxes posterior vocal ligament while maintaining tension of anterior part
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Cricothyroid innervation
External laryngeal nerve (branch of CNX)
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Innervation of intrinsic muscles of larynx (expert cricothyroid)
Recurrent laryngeal nerve (branches of CNX) also called inferior laryngeal nerve - the terminal branches of recurrent laryngeal
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Where are intercostal muscles found
Between each rib
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Function of iC muscles
Bridge gaps between ribs and stop any air or pressure transfer
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Layers of IC muscles
External Ix are found outside then internal IC | Transverse thoracic muscles are innermost
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What is in the IC neuromuscular bundle
IC vein, artery and nerve (superior --> inferior) | Sits between internal IC and innermost IC
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How does the structure of the external IC change
Gets thinner laterally
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Muscle attachment of SCM
Runs obliquely from mastoid process down to sternum | Attaches to manubrium and clavicle (lateral boundary of triangle)
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SCM
Sternocleoimastoid
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Inferior border of anterior triangle of neck
Manubrial notch
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Superior border of anterior triangle of neck
Lower border of mandible
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Midline of anterior triangle of neck
Midline of neck separates R and L
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Where are the infrahyoid muscles found
Deep to SCM - run from hyoid bone to clavicle of scapula
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What does the contraction of suprahyoid muscles cause
The hyoid bone to be pulled up
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What does the contraction of the infrahyoid muscles cause
Hyoid bone to be pulled down
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What is the laryngeal prominence attach dto
Thyroid cartilage
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What is under the laryngeal prominence
Cricoid cartilage
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Where do the vocal cords lie
Deep in thyroid cartilage
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Where is the cricothyroid membrane found
In between thyroid and cricoid cartilage
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What structures lie deep to SCM
Anterior jugular veins | Common carotids, internal jugular and vagus nerve - carotid sheath
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What does the parietal pleura line
Internal surface of thoracic cavity, diaphragm and mediastinum
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What does the visceral pleura cover
Surface of lungs
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Cervical pleura
Parietal pleura in cervical region
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Costal pleura
Parietal pleura in thoracic region
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Diaphragmatic pleura
Parietal pleura covering diaphragm
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Mediastinal pleura
Parietal pleura covering mediastinum
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What is the diaphragmatic pleura innervated by
Phrenic nerves
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What is the costal pleura innervated by
IC nerve
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What is the cervical pleura innervated by
Upper IC nerve and cervical plexus
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What is the mediastinal pleura innervated by
Phrenic nerves
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Why is the pain from the visceral pleura dull
Visceral is innervated autonomically not somatically
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What type of pleura causes radiating pain
Dipahragmatic or mediastinal - referred pain at shoulder | Costal pain is v localised
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Where do the phrenic nerves pass from
C3,4 & 5, pass through neck, over anterior scalene and descend on R and L side of mediastinum.
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Why is the diaphragm higher on the R side
Due to liver
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If there is a build up of fluid in the thoracic cavity, where will fill up
Costa-diaphragmatic recess - visible on CXR
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Anterior attachment of diaphragm
Posterior aspect of diploid process
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Lateral attachment of diaphragm
Costal cartilages of lower ribs (T7-T10)
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Posterior attachment of diaphragm
Arcuate ligament and lumbar vertebrae via crura
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Mpvemen of diaphragm in breathing
Up in inspiration and down in expiration
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What can agitation to diaphragm result in
Referred pain - shoulder or jaw pain
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How long do you have to use ICS to see results
8-10 days of continued use
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What is the peak flow variability threshold
20%
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What is the peak flow reversibility threshold
20%
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3 main causes of COPD
Rhinitis/ PND Asthma GORD
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Taking spirometry measurements
Ask pt to sit up, legs uncrossed Need to do 3 relaxed blows and 3 forceful For the relaxed blows, the pt needs to hold their nose There needs to be a proper seal around mouthpiece
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How should inhalers be taken
Puff needs to be coordinated w/ inspiration
325
Examples of MART drugs
LABA/ICS inhaler: Duoresp Fostair Symbicort
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How often is COPD reviewed
Annually
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Normal spirometer readings
FEV1 > 80% FVC > 80% Ratio > 0.7
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Obstructive spirometer readings
FEV1 < 80% FVC <80% or >80% Ratio < 0.7
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Restrictive spirometer readings
FEV1 < 80% FVC < 80% Ratio > 0.7 (both reduced)
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On which side would you see a gastric air bubble on a CXR
L - stomach
331
What are signs of a raised diaphragm on a CXR
<6 ribs visible anteriorly
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Sign of hyperinflation on CXR
If >8 ribs visible
333
When might you see surgical emphysema
During a PTX drain - air travels elsewhere Stab wounds Airbubble on CXR
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Percussion in pneumonia pts
Dullness
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Auscultation in pneumonia pts
Crackles Bronchial breathing Increased vocal resonance
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Percussion in pleural effusion
Stony dullness
337
Auscultation in pleural effusion pas
Decreased vocal resonance | Decreased breath sounds
338
Percussion in PTX pts
Hyper resonant
339
Auscultation in PTX pts
Absent breath sounds | Decreased breath sounds
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Percussion in lung collapse pts
Dullness
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Auscultation in lung collapse pts
Decreased breath sounds | Decreased VR
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In which lobes is collapse most common
Upper
343
What should you suspect if you see hypercalcaemia in resp pts
Sarcoidosis or cancer
344
What condition is V/Q scan most indicated in
PE
345
What is lymphoma associated with on a CXR
Mediastinal node enlargement
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Types of causes of fibrotic lung disease
``` Lung damage: infarction, pneumonia, TB Irritants: Coal dust, silica DPLD: IPF, HS pneumonitis CTD: RhA, SLE, SScl Meds - amiodarone, nitrafurantoin ```
347
What kind of granuloma is seen in sarcoidosis
``` Non caeseating (non necrotising) Also seen in HS pneumonitis ```
348
Pathophysiology of resp failure
Perfusion of a part of the lung is subjected to impaired ventilation causing hypoxic and CO2 containing blood entering the pulmonary vein
349
Causes of Type 1 a/c resp failure
``` A/c asthma Pulmonary oedema Pneumonia Lobar collapse PTX PE ARDS ```
350
Causes of Type II a/c resp failure
``` A/c severe asthma A/c exacerbation of COPD Upper airway obstruction Central depression Muscle weakness ```
351
Ix for a/c resp fsilure
``` ECG CXR Bloods Dipstick ABGs ```
352
Mx of a/c resp failure - Type 1
High conc of oxygen (40-60%) by mask Mechanical ventilation will be needed to relieve hypoxia Oxygen given should be humidified
353
Mx of Type II a/c resp failure
Emergency - immediate intubation or tracheostomy Treat cause Supported ventilation may be needed IF responsive
354
Most common cause of 'a/c on c/c' type II resp failure
Severe COPD - exacerbations
355
In which zone is asbestosis usually found
Lower zones
356
UIP pattern
Fibroblastic infiltrates Mature fibrosis Honey combing - end stage fibrosis Histological + radiological pattern, not separate disease
357
What zone does HS pneumonitis usually affect
Upper
358
When do we see BHL on a CXR
Sarcoidosis TB Lymphoma
359
When do we see reticular shadowing on a CXR
Infection Cancer ILD
360
Why does hypercapnia cause resp acidosis
Formation of carbonic acid
361
Compensation of resp acidosis cased by increased pCO2
Increase in HCO3-
362
Compensation for metabolic acidosis caused by decreased HCO3-
Decreased CO2
363
Stages of treatment for Type 2 resp failure;ure
Reduce oxygen and recheck ABG (if over oxygenated) NIV if still in resp failure Invasive mechanical ventilation Palliate - respect form
364
What causes finger nail clubbing
C/c hypoxaemia
365
Honeycombing vs ground glass appearance
Honeycombing is spp for end stage ILD | Ground glass is more mild and not spp
366
Appearance description of UIP on CT
Honeycombing | Traction bronchiectasis
367
Appearance description of NSIP
Ground glass
368
When is UIP pattern seen the most
IPF or RhA
369
When is NSIP pattern on CT seen most commonly in
CTD - ILD | Except RhA
370
What pathologies will cause a multiple ill-defined opacities in the lungs
``` Pulmonary infarcts Pulmonary metastases RhA Granulomatosis w/ polyangitiis Septic emboli ```
371
Why do we see bilateral parotid swelling in sarcoidosis
Lymphomatous infiltration
372
Causes of respiratory alkalosis
``` CNS infection SAH Panic attack Aspirin overdose PE (reflex hyperventilating) Anaemia ```