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