Neonatal Lupus Flashcards

1
Q

Anti-Ro60/Trove2

A

60-kD polypeptide

  • Ro60 protects the cell from damage from UV
  • play a role in RNA quality control
  • May protect against the development of autoabs by sequestering defective RNPs
  • Can appear on the cell surface during stress or apoptosis and therefore be a direct target for autoantibodies
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2
Q

Anti La/48

A

Can appear on the cell surface during stress or apoptosis and therefore be a direct target for autoantibodies
- Not a good screening tool alone

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3
Q

Anti Ro 52/Trim 21

A

Catalyzes the ubiquitination of
several proteins including Ro 52 itself
- Regulates of proinflammatory cytokine production
- The production of type I ifn and cytokine via its ubiquitination of IRF
- Regulation of innate immune response to dsDNA
- Modulation of follicular b cell homeostasis and IG production
- Less sensitive but more specific (found in 70-100 percent of mothers of children with CHB

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4
Q

Autoantibodies to test for in NLE

A
  • Anti Ro60
  • Anti Ro52
  • Anti La 48
  • Anti U1RNP
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5
Q

Most common autoimmune diseases present in a mother of a child with NLE

A

SS
SLE
UCTD

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6
Q

Genes important for the production of anti-Ro and anti-La antibodies

A

HLA DR

HLA DQ

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7
Q

Genes associated with anti Ro52 and anti La48

A

HLA DR3

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8
Q

Risk factors for CHB

A

Mother with high anti-Ro Titers who already had a child with CHB (risk rises from 1 to 5% to 18-25%)

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9
Q

Characteristic pathological finding

A

Absence of degeneration of the AV node with or without the bundle of HIS with replacement by fibrosis and calcification

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10
Q

Risk factors associated with perinatal mortality

A
  • Earlier gestational age at CHB diagnosis
  • fetal hydrops
  • myocarditis
  • EFE
  • CHB with a heart rate less than 50 bpm
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11
Q

Treatments for NLE (mother)

A
  • Dexamethasone for second or third degree heart block without EFE, EFE, or EFE with any degree of heart block
  • Maternal IVIG if there is evidence of myocarditis, EFE or incomplete AV block
  • High dose maternal beta 1 adrenergic stimulation is used when fetal heart rates are below 50 bpm or when there is significantly reduced cardiac contractility
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12
Q

Treatments for NLE (Baby)

A
  • Pulse for neonates with myocarditis/EFE
  • A trial of red for neonates with second degree heart block, those who were in CHB in utero and showed signs of improvement and or those who had evidence of myocarditis/EFE on pre or post natal cardiac US
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13
Q

Why is the rash of NLE more common in females?

A

Estrogen increases surface expression of Ro an LA on keratinocytes

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14
Q

Rash associated with NLE

A
  • Resembles lesions of SCLE, less malar , less indurated\
  • Face and scalp are the most commonly involved areas but it can be anywhere including palms and soles
  • Can see raccoon eye distribution
  • Can be present at birth but more commonly develops within the first few weeks of life
  • rarely develop after 6 months
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