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
Role of opioids in fibromyalgia?
No role - cause it to be much worse
Fibromyalgia - diagnostic points of tenderness?
Upper outer quadrants of gluteal region
Bilateral mid point of trapezius muscle
CCP - citrullination means what
CCP antibodies a/w what?
peptidylarginine to peptidylcitrulline
Severe destructive RA, RA-ILD + CVD
3 mucosal sites a/w Rheumatoid arthritis
Lungs - Smoking
Oral periodontal disease - PADI
GI tract
PADI function in RA?
PADI gene from porphyromonas gingivalis - converts arginine to citrulline
what is RS3PE syndrome
Rheumatoid seronegative symmetric synovitis with pitting edema
- boxing glove and shoes appearance - tendosynovitis
- excellent response to steroids
Importance of eye manifestations in RA?
Rare but very high likelihood of Rheumatoid arthritis - RF/CCP positive
-uveitis/episcleritis/anterior chamber eye perforation
Rheumatoid nodules in RA importance?
what are they?
Rheumatoid nodules = Poor prognosis
Nodules = terminal vasculitis with central necrosis and surrounding fibroblasts
Greatest sensitivity for RA - what imaging?
Ultrasound
How long to monitor RA radiographic progression?
5 years
When to start biologics for RA?
When tried 2 or more conventional DMARDS for at least 6 months
Conventional DMARDS side effects?
- MTX
- Sulfalazine
- Hydrochloroquine
- Leflunomide
- Tofacitinib
- TNF inhibitors?
MTX - oral ulcers/ bone marrow toxicity/hepatotoxicity
Sufalazine - agranulocytosis/ azoospermia
HCQ - Retinopathy/blue-grey skin discoloration/photosensitivity
Leflunomide - pneumonitis/peripheral neuropathy
Tofacitinib - Reactivation TB/Herpes Zoster
TNFi - Drug induced lupus/Demyelinating syndrome/Heart failure
Biologics DMARDS side effect
TNFi - Infliximab/Adalimumab
Anti-CD20 - Rituximab ( best for seropositive RA)
IL6i - Tocilizumab
Rituximab - PML/reactivation of TB
Tocilizumab - Diverticular perforation/reactivation TB
Prednisolone role in RA?
short-term Bridge till DMARDS efficacy achieved
Takayasu arteritis likes to affect which arteries?
Which HLA it is associated most with?
Aorta - 1st ( can have occlusion)
Renal arteries - 2nd
HLA B51
Clinical features of Takayasu arteritis?
- Claudication of extremities
- Brachial arteries = decrease pulsation
- Difference at least 10 mmHg systolic blood pressure between arms
- Bruits - abdominal aorta/ subclavian arteries
