Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute

林 聖傑(Sheng-Chieh Lin), 徐 世達(Shyh-Dar Shyur), 朱 斯鴻(Szu-Hung Chu), 黃 立心(Li-Hsin Huang), 吳 俊誼(Jiunn-Yi Wu), 馬 益群(Yi-Chun Ma)

Research output: Contribution to journalArticle

Abstract

Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants.
Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003.
Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block.
Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.
Original languageEnglish
Pages (from-to)11-22
Number of pages12
JournalFormosan Journal of Rheumatology
Volume18
Issue number3&4
Publication statusPublished - 2004
Externally publishedYes

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Mothers
Antibodies
Cutaneous Lupus Erythematosus
Skin
Heart Block
Atrioventricular Block
Central Nervous System Diseases
Cholestasis
Serology
Transaminases
Neonatal Systemic lupus erythematosus
Autoantibodies
Placenta
Autoimmune Diseases
Anemia
Pregnant Women
Anti-Idiotypic Antibodies
Heart Diseases
Seizures
Congenital heart block

Keywords

  • Neonatal lupus erythematosus
  • telangiectasla
  • congenital complete heart block
  • cholestatic hepatitis
  • focal seizure

Cite this

林聖傑(Sheng-Chieh L, 徐世達(Shyh-Dar S, 朱斯鴻(Szu-Hung C, 黃立心(Li-Hsin H, 吳俊誼(Jiunn-Yi W, & 馬益群(Yi-Chun M (2004). Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute. Formosan Journal of Rheumatology, 18(3&4), 11-22.

Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute. / 林聖傑(Sheng-Chieh Lin); 徐世達(Shyh-Dar Shyur); 朱斯鴻(Szu-Hung Chu); 黃立心(Li-Hsin Huang); 吳俊誼(Jiunn-Yi Wu); 馬益群(Yi-Chun Ma).

In: Formosan Journal of Rheumatology, Vol. 18, No. 3&4, 2004, p. 11-22.

Research output: Contribution to journalArticle

林聖傑(Sheng-ChiehL, 徐世達(Shyh-DarS, 朱斯鴻(Szu-HungC, 黃立心(Li-HsinH, 吳俊誼(Jiunn-YiW & 馬益群(Yi-ChunM 2004, 'Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute', Formosan Journal of Rheumatology, vol. 18, no. 3&4, pp. 11-22.
林聖傑(Sheng-ChiehL, 徐世達(Shyh-DarS, 朱斯鴻(Szu-HungC, 黃立心(Li-HsinH, 吳俊誼(Jiunn-YiW, 馬益群(Yi-ChunM. Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute. Formosan Journal of Rheumatology. 2004;18(3&4):11-22.
林聖傑(Sheng-Chieh Lin) ; 徐世達(Shyh-Dar Shyur) ; 朱斯鴻(Szu-Hung Chu) ; 黃立心(Li-Hsin Huang) ; 吳俊誼(Jiunn-Yi Wu) ; 馬益群(Yi-Chun Ma). / Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute. In: Formosan Journal of Rheumatology. 2004 ; Vol. 18, No. 3&4. pp. 11-22.
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title = "Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute",
abstract = "Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants.Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003.Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block.Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.",
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author = "林, {聖傑(Sheng-Chieh Lin)} and 徐, {世達(Shyh-Dar Shyur)} and 朱, {斯鴻(Szu-Hung Chu)} and 黃, {立心(Li-Hsin Huang)} and 吳, {俊誼(Jiunn-Yi Wu)} and 馬, {益群(Yi-Chun Ma)}",
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TY - JOUR

T1 - Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute

AU - 林, 聖傑(Sheng-Chieh Lin)

AU - 徐, 世達(Shyh-Dar Shyur)

AU - 朱, 斯鴻(Szu-Hung Chu)

AU - 黃, 立心(Li-Hsin Huang)

AU - 吳, 俊誼(Jiunn-Yi Wu)

AU - 馬, 益群(Yi-Chun Ma)

PY - 2004

Y1 - 2004

N2 - Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants.Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003.Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block.Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.

AB - Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants.Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003.Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block.Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.

KW - Neonatal lupus erythematosus

KW - telangiectasla

KW - congenital complete heart block

KW - cholestatic hepatitis

KW - focal seizure

M3 - Article

VL - 18

SP - 11

EP - 22

JO - Formosan Journal of Rheumatology

JF - Formosan Journal of Rheumatology

SN - 2075-0374

IS - 3&4

ER -