SCLE- like syndromes Flashcards

1
Q

What is pathogenesis of neonatal lupus?

A

transplacental passage of maternal autoantibodies, most importantly Ro/SSA

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2
Q

What is a major concern for baby with neonatal lupus?

A

autoantibodies can result in heart block requiring pacemaker

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3
Q

What is risk of mom with SLE to have a baby with neonatal lupus? What if she previously had a baby with neonatal lupus?

A
  • 15%
  • 25% if prior baby with NLE
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4
Q

When do skin lesions from neonatal lupus appear?

A

usually within first few weeks of life, but not present at birth

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5
Q

What are the clinical features of neonatal lupus?

A
  • similar lesions to adults with SCLE but more prominent facial involvement
  • raccoon eyes from periorbital erythema
  • photosensitivity

- annular, polycyclic, erythematous plaques w/ central clearing and raised red border, fine scale

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6
Q

how do lesions of neonatal lupus heal?

A

non scarring with dyspigmentation and telangiectasias

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7
Q

What percent of patients with neonatal lupus will have some cardiac abnormality? What % have congenital third degree heart block?

A
  • 70% have some abnormality

30-40$ have third degree heart block

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8
Q

When does heart block present in neonatal lupus?

A

at birth almost always

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9
Q

How does neonatal lupus heartblock present?

A

bradycardia and irreversible complete heart block at birth

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10
Q

Besides heartblock, what systemic findings should you check for in neonatal lupus?

A
  • Hepatobiliary disease (transient hyperbilirubinemia or transient elevated liver enzymes)

- Hematologic (thrombocytopenia, neutropenia, lymphopenia, hemolytic anemia)

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11
Q

What % of women who give birth to baby with neonatal lupus are asymptomatic at time of childs birth?

A

50% have no symptoms of SLE

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12
Q

Is there any way to decrease risk of heart disease in neonatal lupus?

A

- prenatal systemic corticosteroids

- hydroxychloroquine throughout pregnancy

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13
Q

What is clinical course/prognosis of these factors in neonatal lupus:

  • skin disease
  • cardiac disease
  • hematologic/LFT abnormalities
A
  • skin disease resolve without scarring by ~6 months (may have residual atrophy, dyspigmentation and telangiectasias for months to years)
  • Third degree block is irriversible and requires lifelong pace-maker, but lesser blocks may resolve on own.
  • Heme and LFT abnormalities spontaneously resolve within 4-6 months
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14
Q

What is the most common cause of complement deficiency-associated SLE?

A

C2 deficiency

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15
Q

What % of patients with C2 complement deficiency will develop SLE?

A

only 10-20%, but because it is the most common complement deficiency, this is the most frequent deficiency associated with SLE

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16
Q

Which complement deficiencies have the highest risk of being associated with SLE?

A

C1q and C4 (these are very rare, so still the most common cause of complement deficiency related SLE is C2 deficiency- despite the lower risk of getting SLE in those patients)

17
Q

What is pathogenesis of complement-deficiency associated SLE?

A
  • complement usually helps clear apoptotic bodies that have high levels of autoantigens like Ro/SSA. this leads to loss of immune tolerance and autoantibody-mediated inflammation
18
Q

How does C2 deficiency-associated SLE present?

A

like SLE but with less severe systemic disease (mild or absent renal disease)

  • prominent photosensitivity and SCLE lesions
  • increased bacterial infections w/ encapsulated bacteria
19
Q

C2 deficiency associated SLE patietns are at increased risk for what type of infections?

A
  • encapsulated bacteria (especially strep pnuemonia)
20
Q

How does C1 and C4 deficiency associated SLE present?

A
  • severe recalcitrant renal disease
  • photosensitivity with CCLE or SCLE
  • palmoplantar keratoses in C4 deficiency
  • increased risk of infection with encapsulated bacteria and candida