Respiratory Flashcards
Classification of ILD
ILD known cause
Granulomatous
Other forms (pLAM)
Idiopathic, interstitial pneumonias
Mosaic ventilation, segmental air trapping
Starry sky nodules
Hypersensitivity pneumonitis
ILD Associated with tuberous sclerosis and cysts on CT chest
pLAM
Forms of ILD assocaited with smoking, and can be reversed by stopping smoking
RB-ILD
DIP (desquamative)
Idiopathic ILD most response to steroids
COP
ILD mimicking ARDS, worst prognosis
AIP
ILD with peri-bronchovascular cysts (cysts next to dilated bronchi)
LIP - lymphocytic interstitial pneumonia
Idiopathic ILD most commonly associated with autoimmune disease
NSIP
Features of UIP
Honeycombing
Subpleural reticulation
Basal predominance
Traction bronchiectasis
4 diffuse cystic lung diseases
pLAM
Langerhan’s cell histiocytosis
LIP
Birt Hogg Dube syndrome
Cystic lung disease associated with tuberous sclerosis
Often has FHx
pLAM
Cystic lung disease associated with smoking
Langerhans cell histiocytosis
Cystic lung disease with FHx and associated with CTD (particularly Sjogren’s)
LIP
Cystic lung disease with FHx of pneumothorax
Birt Hogg Dube syndrome
Nintedanib MoA and common A/E
MoA - blocks multiple TKI’s (VEGF, PDEGF
A/E - Diarrhoea
Contraindiacted in severe liver impairment
Pirfenidone MoA and common A/E
MoA - antifibrotic, inhibiting TGFb and fibroblast proliferation
A/E - Drug induced liver injury
UIP radiology features
Honeycombing
Subpleural reticulation
Basal predominance
Traction bronchiectasis
NSIP radiology features
Ground glass changes
Subpleural sparing
1st and 2nd line Treatment of OHS
OHS and OSA (90%) - CPAP
No OSA, and sleep hypoventilation - BiPAP
2nd line - BiPAP
Treats narcolepsy but not cataplexy
Modafanil - 1st line in narcolepsy
Good treatment of cataplexy
Sodium oxybate
Methylphenidate
REM sleep disorder features and significance
Intrusion of wakelfullness in REM –> lack of atonia in REM sleep
Predates dementia by 10 years, strongly associated with synucleiopathies
Hypersomnia
Hyperphagia
Hypersexuality
Klein Levin syndrome
Most common pattern ILD in RA
UIP
A/E of nitrofurantoin
Pulmonary fibrosis
Diffuse pulmonary infiltrates
Acute
Eosinophilia
Rapid response to steroids
Eosinophilic pneumonia
CFTR gene -which chromosome?
7
Severe CF characterised by?
What classes of CFTR mutation fit this?
Reduce/absent production of CFTR, or reduced CFTR reaching membrane
1 - absent production
5 - reduced production
2 - reduce amount reaching membrane (FD508)
Most common CF allele
Delta F508
Ivakaftor
- Moa
- Uses
Binds to defective CFTR to increase function
Use in class 3 (defective regulation) and class 4 (defective travel of ions).
1st line for CF with delta F508 (homozygote and heterozygote)
- > 6
- < 6
> 6 - trikafta
< 6 - orkambi
Mutation causing defective transport of CFTR protein to membrane
- name
- class
Delta F508
Class 2
MoA of Evusheld
Pre-exposure Ppx
Recombinant IgG binds to S spike protein, prevents binding to ACE receptor and entry into host cell
CI to paxlovid
CPC cirrhosis
eGFR < 30
On other medications metabolised by CYPS
>5 days symptoms
Nirmeltravir MoA
Inhibits COVID protease - prevents polyprotein processing and viral replication
COVID therapy safe in pregnancy
Remdesevir
Timeline within to use remdes
< 7 days
MoA remdesevir
Inhibits RNA dependant RNA polymerase (forms adenosine triphoshates that competitively inhibit)
MoA of baricitinib
Janus kinase 1 and 2 inhibitor
Pulm HTN and pregnant
- Which to use
- What is CI
Sildenafil
Bosentan and rociguat contraindicated
Most common form lung cancer
Adenocarcinoma
Upper lobe lung pathology
SET CAP
Siicosis
Extrinsice alveolitis
TB
CF
Ank Spon
PCP
Lung Tx patients with highest survival?
CF - as they are youngest
Warm ischaemic time
- Higher in?
- Outcomes?
Ischaemic time at normal termperature
Higher in DCD (have to wait 60 minutes)
High early graft dysfunction, same survival outcomes
FEV1 to refer for lung transplant in COPD?
< 25%
BODE score
BMI
Obstruction (FEV1)
Dyspnoea (mCRC)
Excercise (6MWT)
3 for FEV1 < 35%
3 for stage 4 mCRC
When to refer for lung transpant based on BODE
BODE 5 or more
When to refer ILD for Lung Tx
UIP of fibrotic NSIp
Any functional or O2 impairment
CI to lung reduction surgery
DLCO < 20
FEV1 < 20
Benefits of lung reduction volume surgery
Overall survival advantage
Reduce hyperinflation
1st line management of CLAD (PPx also)
Azithromcyin
Management for CTEPH class 1/2 NYHA
ricoguat
Most common cause pulmonary hypertension
L heart disease
Rapidly progressive class I PAH
PVOD
CUrative treatment for CTEPH
Endarterctomy - first line if feasible (assess with CTPA)
mCR dyspnoea score
4 - can’t leave house
3 - 100m
2 - Walks slower than someone same age
> 50% VC drop when standing
Dyspnoea when standing
Diaphragmatic palsy
Tests for unilateral diaphragamtic palsy
Sniff test - video fluoro, paradoxical rise
Low MIP
Direct bronchoprovocation
Histamine, metacholine
Indirect bronchoprovocation
Hypertonic saline, mannitol
Benefit of bronchprovocation
High NPV
Rule out asthma
High FeNO?
Measure of steroid hyperresponsiveness
Value of CPET
Can differentiate if dyspnoea is due to ventilation, pulmonary, cardiac or metabolic
Bronchiectasis - treatment to prevent exacerbations
Long term azithro
Aspergillus in sputum - when is it of consequence?
Any one immunosuppressed
Immunocompetent - both fungal elements and hyphae on stain
Screening test for Kartegeners
Low NO exhalation
Diagnosis of CF
FHX of clinic features
AND 1 of:
- x2 positive sweat tests
- 2 CF causing mutations
- Nasal PD tracing typical for CF
Best way to test compliance with CFTR modulators
Nasal potential difference assessment
Differentiate between acute and chronic eosinophilic pneumonia
Both are rapidly responsive to steroids
Acute will NOT have peripheral eosinophila because they are all in the lungs
Light’s for exudative
Protein - pleural > 50%
LDH - Pleural > 60%
Pleural LDH > 0.45
Pleural fluid suggestive of empyema
ph < 7.2
Microorganism on gram stain
Pleural glucose < 40
Decreased in O2 sats on CPET
Pulmonary vascular disease
Muscles of inspiration
External intercostals
Diaphragm
Muscle of expiration
- Passive
- Forces
Recoil of lungs and diaphragam
Abdominal muscles, internal intercostals
Which lung volume is PVR lowest?
FRC
When is D Dimer useful
If PE pre-test probability is low
Can rule out diagnosis
MoA pirfenidone
Inhibits TGF-b
Main A/E pirfinedone
Rash, photosensitivity
MoA nintendanib
TKI for VEG/PDGF
A/E of nintendanib
LFT derangement, diarrhoea
CI to nintendanib
CPB and CPC cirrhosis
CYP3A4 and p glycoprotein inducers
Which hormone stimulates respiration
Progesterone
Methoprogesterone used for sleep disordered breathing
Most important peripheral chemoreceptor
Carotid bodies - detect PaO2 and increase ventilation
Central chemoreceptors detect?
pH as a product of CO2
Reason for improved excercise capacity in lung reduction
Improved elastic recoil