Neonatal Lupus Erythematosus
Related Subjects:
| Systemic Lupus Erythematosus (SLE)
| Drug-induced Lupus Erythematosus
| Discoid Lupus Erythematosus (DLE)
| Neonatal Lupus Erythematosus
👶 Neonatal Lupus Erythematosus (NLE) is a rare autoimmune condition in infants of mothers carrying anti-Ro/SSA or anti-La/SSB antibodies.
Some mothers have known SLE/Sjögren’s, while others are asymptomatic carriers.
🧬 Aetiology
- Maternal IgG anti-Ro (SSA) and anti-La (SSB) antibodies cross the placenta.
- Bind to fetal tissues → inflammation and damage.
🩺 Clinical Effects
- ❤️ Cardiac: Congenital heart block (any degree) → bradycardia ± heart failure; may progress to cardiomyopathy or endocardial fibroelastosis.
- 🌸 Skin: Circular/elliptical erythematous rash, often periorbital (“raccoon eyes”); triggered by UV exposure.
- 🩸 Haematological: Haemolytic anaemia, thrombocytopenia, neutropenia.
- 🧪 Hepatic: Raised LFTs, hepatomegaly.
🔍 Investigations
- Maternal: Anti-Ro/La antibody screen; assess for SLE/Sjögren’s.
- Prenatal: Serial fetal echocardiography from 16 wks; fetal heart monitoring for bradycardia.
- Neonatal: ECG + echo; FBC; LFTs; ± skin biopsy if uncertain.
⚕️ Management
- Prenatal: Close monitoring with scans; hydroxychloroquine may reduce risk; steroids only if specialist-guided.
- Postnatal:
- Cardiac: Pacemaker for complete block; heart failure therapy; lifelong cardiology follow-up.
- Skin: Avoid sun; protective clothing; topical steroids if needed. Lesions usually resolve as antibodies clear.
- Haematological: Monitor counts; transfusion if severe anaemia; manage neutropenia infections.
- Hepatic: Supportive, usually self-resolving.
- Family support: Education, prognosis, genetic counselling for future pregnancies.
📈 Prognosis
- Skin, liver, and blood features resolve by 6–8 months (maternal antibody clearance).
- Congenital heart block is usually permanent → pacing + lifelong cardiology care.
- With early diagnosis + support, many infants lead healthy lives.
📚 References
