SCLE- like syndromes

Question 1

What is pathogenesis of neonatal lupus?

Answer 1

transplacental passage of maternal autoantibodies, most importantly Ro/SSA

Question 2

What is a major concern for baby with neonatal lupus?

Answer 2

autoantibodies can result in heart block requiring pacemaker

Question 3

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

Answer 3

• 15%
• 25% if prior baby with NLE

Question 4

When do skin lesions from neonatal lupus appear?

Answer 4

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

Question 5

What are the clinical features of neonatal lupus?

Answer 5

• 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

Question 6

how do lesions of neonatal lupus heal?

Answer 6

non scarring with dyspigmentation and telangiectasias

Question 7

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

Answer 7

• 70% have some abnormality

30-40$ have third degree heart block

Question 8

When does heart block present in neonatal lupus?

Answer 8

at birth almost always

Question 9

How does neonatal lupus heartblock present?

Answer 9

bradycardia and irreversible complete heart block at birth

Question 10

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

Answer 10

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

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

Question 11

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

Answer 11

50% have no symptoms of SLE

Question 12

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

Answer 12

- prenatal systemic corticosteroids

- hydroxychloroquine throughout pregnancy

Question 13

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

• skin disease
• cardiac disease
• hematologic/LFT abnormalities

Answer 13

• 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

Question 14

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

Answer 14

C2 deficiency

Question 15

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

Answer 15

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

Question 16

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

Answer 16

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)

Question 17

What is pathogenesis of complement-deficiency associated SLE?

Answer 17

• 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

Question 18

How does C2 deficiency-associated SLE present?

Answer 18

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

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

Question 19

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

Answer 19

• encapsulated bacteria (especially strep pnuemonia)

Question 20

How does C1 and C4 deficiency associated SLE present?

Answer 20

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