Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 124-130 |
| Number of pages | 7 |
| Journal | Japanese Journal of Clinical Immunology |
| Volume | 40 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 2017 Jan 1 |
| Externally published | Yes |
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Keywords
- Anti-SS-A antibody
- Congenital heart block
- Hydroxychloroquine
- Neonatal cutaneous lupus erythematosus
- Neonatal lupus erythematosus
ASJC Scopus subject areas
- Immunology and Allergy
- Immunology
Cite this
Neonatal lupus erythematosus. / Yokogawa, Naoto; Sumitomo, Naofumi; Miura, Masaru; Shibuya, Kazuhiko; Nagai, Hiroshi; Goto, Mikako; Murashima, Atsuko.
In: Japanese Journal of Clinical Immunology, Vol. 40, No. 2, 01.01.2017, p. 124-130.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Neonatal lupus erythematosus
AU - Yokogawa, Naoto
AU - Sumitomo, Naofumi
AU - Miura, Masaru
AU - Shibuya, Kazuhiko
AU - Nagai, Hiroshi
AU - Goto, Mikako
AU - Murashima, Atsuko
PY - 2017/1/1
Y1 - 2017/1/1
N2 - 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.
AB - 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.
KW - Anti-SS-A antibody
KW - Congenital heart block
KW - Hydroxychloroquine
KW - Neonatal cutaneous lupus erythematosus
KW - Neonatal lupus erythematosus
UR - http://www.scopus.com/inward/record.url?scp=85020642421&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85020642421&partnerID=8YFLogxK
U2 - 10.2177/jsci.40.124
DO - 10.2177/jsci.40.124
M3 - Article
C2 - 28603203
AN - SCOPUS:85020642421
VL - 40
SP - 124
EP - 130
JO - Immunological Medicine
JF - Immunological Medicine
SN - 0911-4300
IS - 2
ER -