A Case of Neonatal Lupus Erythematosus Presenting Delayed Dilated Cardiomyopathy With Circulating Autoantibody to Annexin A6

Yoshinori Seko; Akihiro Matsumoto; Taira Fukuda; Yasushi Imai; Tsutomu Fujimura; Hikari Taka; Reiko Mineki; Kimie Murayama; Yasunobu Hirata; Ryozo Nagai

doi:10.1536/ihj.48.407

Case Reports

A Case of Neonatal Lupus Erythematosus Presenting Delayed Dilated Cardiomyopathy With Circulating Autoantibody to Annexin A6

Yoshinori Seko, Akihiro Matsumoto, Taira Fukuda, Yasushi Imai, Tsutomu Fujimura, Hikari Taka, Reiko Mineki, Kimie Murayama, Yasunobu Hirata, Ryozo Nagai

Author information

Yoshinori Seko
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo
Akihiro Matsumoto
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo
Taira Fukuda
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo
Yasushi Imai
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo
Tsutomu Fujimura
Division of Proteomics and Biomolecular Science, BioMedical Research Center, Graduate School of Medicine, Juntendo University
Hikari Taka
Division of Proteomics and Biomolecular Science, BioMedical Research Center, Graduate School of Medicine, Juntendo University
Reiko Mineki
Division of Proteomics and Biomolecular Science, BioMedical Research Center, Graduate School of Medicine, Juntendo University
Kimie Murayama
Division of Proteomics and Biomolecular Science, BioMedical Research Center, Graduate School of Medicine, Juntendo University
Yasunobu Hirata
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo
Ryozo Nagai
Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo

Keywords: 2-D gel electrophoresis, Anti-SS-A/-SS-B antibody, β-blocker, Ca²⁺ transient, Congenital heart block, Heart failure, Mass spectrometry, Pacemaker implantation, Western blot

JOURNAL FREE ACCESS

2007 Volume 48 Issue 3 Pages 407-415

DOI https://doi.org/10.1536/ihj.48.407

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Abstract

Patients with neonatal lupus erythematosus (NLE) often have congenital heart block with or without heart failure and are born to mothers who have anti-SS-A and/or anti-SS-B antibodies. NLE has been considered to result from the placental transmission of maternal autoantibodies into the fetal circulation causing myocardial damage. We report a case of NLE with congenital heart block who had undergone pacemaker implantation at the age of 17, and then developed dilated cardiomyopathy (DCM) at the age of 19, which is much later than in most other cases. The patient's mother was positive for anti-SS-A and anti-SS-B antibodies, whereas the patient was negative for both anti-SS-A and anti-SS-B antibodies. There were some autoantibodies against cell surface antigens of cardiac myocytes in the serum from the patient, and annexin A6 was identified as one of the autoantigens. This is the first report demonstrating that annexin A6 is involved in the myocardial injury in patients with NLE. The results indicate that inhibition of annexin A6 function may prevent autoantibody-mediated myocardial injury in at least some cases of DCM.

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