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- YOKOGAWA Naoto
- Tokyo Metropolitan Tama Medical Center, Department of Rheumatology
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- SUMITOMO Naofumi
- Tokyo Metropolitan Children's Medical Center, Department of Cardiology
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- MIURA Masaru
- Tokyo Metropolitan Children's Medical Center, Department of Cardiology
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- SHIBUYA Kazuhiko
- Tokyo Metropolitan Children's Medical Center, Department of Cardiology
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- NAGAI Hiroshi
- Kobe University Graduate School of Medicine, Division of Dermatology, Department of Internal Related
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- GOTO Mikako
- National Center for Child Health and Development, The Japan Drug Information Institute in Pregnancy
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- MURASHIMA Atsuko
- National Center of Child health and Development, Division of Maternal Medicine, Department of Perinatology
Bibliographic Information
- Other Title
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- 新生児ループス
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Abstract
<p> Neonatal lupus (NL), a passively-acquired autoimmune disease associated with maternal anti-SSA antibody, presents both cardiac manifestations such as cardiac NL and non-cardiac manifestations including rashes, cytopenia, and hepatic abnormalities. Cardiac NL, occurring in 1-2% of anti-SS-A antibody-positive mothers, is a life-threatening complication with a mortality rate of 20% and a pacemaker implantation rate of 70%. In contrast, cutaneous NL, which is more common than cardiac NL, usually resolves in six months. Since half of NL cases occur in asymptomatic mothers, if an infant presents characteristic cutaneous or cardiac manifestations of NL, the mother should be tested for anti-SS-A antibody. In mothers positive for anti-SS-A antibody, the risk of having a child with cardiac NL increases ten-fold and five-fold for a previous child with cardiac NL and cutaneous NL, respectively. A joint American, British, and French retrospective study of NL registries showed that hydroxychloroquine (HCQ) reduced the cardiac NL risk in subsequent pregnancies in mothers who previously had a child with cardiac NL. A prospective open-label study to confirm this effect is being undertaken in the USA. A similar prospective multi-center study will be undertaken in Japan. Establishing a Japanese registry of children with NL and subsequent pregnancies of their mothers will help promote clinical research in NL in Japan.</p>
Journal
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- Japanese Journal of Clinical Immunology
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Japanese Journal of Clinical Immunology 40 (2), 124-130, 2017
The Japan Society for Clinical Immunology
