Background: Although more than 200 cases of neonatal lupus erythematosus (NLE) have been reported, the prognosis, clinical characteristics, and genetic background of the patients are still obscure. Their symptoms seem to vary in different races.
Objective: We had an opportunity to see two clinically different types of NLE. It is important to define the clinical characteristics in Japanese cases and to compare them to caucasian NLE cases reported earlier.
Methods: Sixty Japanese infants with NLE and their mothers reported in Japan were investigated and compared with cases reported from other countries.
Results: Japanese cases were highly associated with anti-SS-A/Ro, anti-SS-B/La, anti-ribonuclear protein, and anti-DNA antibodies. A low frequency of congenital heart block was noted but 8.3% of the cases progressed to systemic lupus erythematosus. HLA-DRw12 was a significantly relative risk in NLE.
Conclusion: The clinical characteristics of Japanese NLE patients were different from those of caucasian patients reported in the literature.