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Neonatal lupus mimicking extra hepatic biliary atresia
Madhuri Kanitkar1, K P V Rohini, Bipin Puri
1Department of Pediatrics, Command Hospital and Armed Forces Medical College, Pune 411 040, India. madhurikanitkar@rediffmail.com
Indian Pediatrics
|December 30, 2004
Summary
Neonatal Lupus Erythematosus (NLE) is a rare condition. This report details a case of NLE with severe cholestasis, linked to maternal and infant anti-SS-A/Ro and anti-SS-B/La antibodies.
Area of Science:
- Pediatrics
- Immunology
- Hepatology
Background:
- Neonatal Lupus Erythematosus (NLE) is a rare autoimmune condition affecting infants.
- NLE commonly presents with congenital heart block, and less frequently, hepatitis.
- Maternal autoantibodies, specifically anti-SS-A/Ro and anti-SS-B/La, are implicated in NLE pathogenesis.
Observation:
- This report describes an uncommon presentation of NLE.
- The infant exhibited severe cholestasis, a significant liver manifestation.
- Both mother and infant tested positive for anti-SS-A/Ro and anti-SS-B/La antinuclear antibodies.
Findings:
- The case highlights a severe cholestatic presentation of Neonatal Lupus Erythematosus.
- Positive maternal and infant anti-SS-A/Ro and anti-SS-B/La antibodies were confirmed.
- This association suggests a potential link between these antibodies and severe liver involvement in NLE.
Implications:
- This case expands the known spectrum of NLE manifestations.
- It underscores the importance of screening for specific autoantibodies in infants with unexplained cholestasis.
- Further research may elucidate the mechanisms linking these antibodies to severe hepatic dysfunction in NLE.