SCLE- like syndromes Flashcards
What is pathogenesis of neonatal lupus?
transplacental passage of maternal autoantibodies, most importantly Ro/SSA
What is a major concern for baby with neonatal lupus?
autoantibodies can result in heart block requiring pacemaker
What is risk of mom with SLE to have a baby with neonatal lupus? What if she previously had a baby with neonatal lupus?
- 15%
- 25% if prior baby with NLE
When do skin lesions from neonatal lupus appear?
usually within first few weeks of life, but not present at birth
What are the clinical features of neonatal lupus?
- 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
how do lesions of neonatal lupus heal?
non scarring with dyspigmentation and telangiectasias
What percent of patients with neonatal lupus will have some cardiac abnormality? What % have congenital third degree heart block?
- 70% have some abnormality
30-40$ have third degree heart block
When does heart block present in neonatal lupus?
at birth almost always
How does neonatal lupus heartblock present?
bradycardia and irreversible complete heart block at birth
Besides heartblock, what systemic findings should you check for in neonatal lupus?
- Hepatobiliary disease (transient hyperbilirubinemia or transient elevated liver enzymes)
- Hematologic (thrombocytopenia, neutropenia, lymphopenia, hemolytic anemia)
What % of women who give birth to baby with neonatal lupus are asymptomatic at time of childs birth?
50% have no symptoms of SLE
Is there any way to decrease risk of heart disease in neonatal lupus?
- prenatal systemic corticosteroids
- hydroxychloroquine throughout pregnancy
What is clinical course/prognosis of these factors in neonatal lupus:
- skin disease
- cardiac disease
- hematologic/LFT abnormalities
- 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
What is the most common cause of complement deficiency-associated SLE?
C2 deficiency
What % of patients with C2 complement deficiency will develop SLE?
only 10-20%, but because it is the most common complement deficiency, this is the most frequent deficiency associated with SLE