title: A case of neonatal lupus erythematosus presenting delayed dilated cardiomyopathy with circulating autoantibody to annexin A6.
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.
authors.affiliation: Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo, Tokyo.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Seko
authors.forename: Akihiro
authors.initials: A
authors.lastname: Matsumoto
authors.forename: Taira
authors.initials: T
authors.lastname: Fukuda
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Imai
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Fujimura
authors.forename: Hikari
authors.initials: H
authors.lastname: Taka
authors.forename: Reiko
authors.initials: R
authors.lastname: Mineki
authors.forename: Kimie
authors.initials: K
authors.lastname: Murayama
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Hirata
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
mesh_terms: D000328:Adult; D017317:Annexin A6 / Q000097:blood / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001706:Biopsy; D015153:Blotting, Western; D002097:C-Reactive Protein / Q000378:metabolism; D002304:Cardiac Pacing, Artificial; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004452:Echocardiography; D015180:Electrophoresis, Gel, Two-Dimensional; D005500:Follow-Up Studies; D006327:Heart Block / Q000150:complications / Q000151:congenital / Q000628:therapy; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D008297:Male; D013058:Mass Spectrometry; D013318:Stroke Volume; D013997:Time Factors
chemical_list: D017317:Annexin A6; D001323:Autoantibodies; D002097:C-Reactive Protein