Rheumatology 3 Flashcards
SLE mutation and associated with familial chilblain lupus ( cutaneous LE)?
TREX mutation
Key immunopathogenesis in SLE?
Loss of self tolerance to autologous-antigens
- low C3/4 resulting in inability to phagocytose apoptotic materials ( increase in apoptotic materials)
- alot of auto-antigens bind with antibodies = immune complexes causing inflammatory cascade
Immune complexes trigger TLR
- B cells activation and differentiation = BAFF/BLyS - B cell proliferation
- IFN activation ( hypomethylation of DNA)
UV light and SLE?
UV triggers keratinocytes apoptosis = cytokine release - hypomethylation of T cell DNA
Hormones in SLE - what hormones increase and decrease?
Estrogen increased in SLE
Androgen/DHEA decrease in SLE
Most common cardiovascular presentation of SLE?
What cardiac valve most affected and pathology?
pericarditis
Mitral valve- Regurgitation
Lupus hepatitis have what antibody?
Ribosomal P antibody
Mitral valve prolapse in SLE a/w what condition as well?
Antiphospholipid syndrome
ANA positive (ANA can be positive in majority of healthy population) what antibody next?
Anti-DFS-70 - if positive means unlikely SLE
Hydroxychloroquine use in SLE?
improve MMF response
Reduce neonatal lupus risk
Belimumab mechanism of action?
Anti BAFF/BLys - causing B cell to commit suicide
Anifrolumab mechanism of action?
Inhibit IFNa/b signalling
Antiphospholipids antibodies - what type of anticoagulation?
Asymptomatic?
Prior thrombosis?
Prior thrombosis + obstetrics complication?
Asymptomatic - aspirin
Prior thrombosis - Therapeutic LMWH till 6 weeks post-partum
Prior thrombosis + obstetric complication - LMWH (prophylactic) + aspirin
Fluorinated steroids eg and why it exists?
Example
Betamethasone
Fluorinated steroids - cross placenta barrier/ penetrate skin better hence more side effects
Role of fluorinated steroids in neonatal lupus?
Use fluorinated steroids + IVIG in neonates with 2nd heart block = prevents progression to Complete HB
Which steroids does not cross placenta barrier?
Dexamethasone