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      Neonatal lupus syndrome: the heart as a target of the immune system


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          Neonatal lupus erythematosus (NLE) is an auto-immune disease related to systemic lupus erythematosus (SLE). Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflamatory reaction in the fetal tissues. NLE is the principal cause of atria-ventricular block, when it is not associated with congenital birth defects. All the clinical studies to date correlate the heart block in NLE with the presence of certain types of circulating maternal antibodies, against the Ro/SSA nuclear proteins, in the serum of the newborn. In this paper we discuss animal models that have been developed by our and others groups to study the participation of the anti-Ro/SSA antibodies in the pathogenesis of the cardiac conduction blockades that occur in NLE.

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          Body Weight Loss during Lactation in Relation to Energy and Protein Metabolism in Standard-Fed Primiparous Sows

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            Protein heterogeneity in the human Ro/SSA ribonucleoproteins. The 52- and 60-kD Ro/SSA autoantigens are encoded by separate genes.

            Two cDNA clones encoding the 52-kD form of a protein present in human Ro/SSA ribonucleoprotein complexes were cloned from a lambda gt11 human thymocyte cDNA library. These clones reacted with lupus patient sera which had anti-52-kD Ro/SSA antibodies, and with affinity-purified anti-52-kD Ro/SSA antibodies. Moreover, affinity-purified antibodies isolated from isopropyl-beta-D-thiogalactopyranoside-induced proteins of these clones reacted only with the 52-kD protein of lymphocytes in Western blots and precipitated Ro/SSA hY RNAs, confirming that the clones encode a 52-kD Ro/SSA antigen. The cDNA contains a single open reading frame of 1,425 nucleotides and encodes a predicted 475-amino acid polypeptide with a molecular mass of 54,108 D. This protein appears unique in that both a zinc finger and leucine zipper motif are present on this protein. Surprisingly, no homology was found between the 52-kD Ro/SSA gene or protein and three published 60-kD Ro/SSA sequences. However, significant similarity of the 52-kD Ro/SSA was detected with human rfp and mouse rpt-1. These three proteins each contain similar zinc finger motifs in approximately their first 145 amino acid residues. The cDNA and the protein expressed therefrom are useful in the analysis of the structural and functional properties of this autoantigen.
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              Anti—Ro/SS-a Antibodies in the Pathophysiology of Congenital Heart Block in Neonatal Lupus Syndrome, An Experimental Model


                Author and article information

                Role: ND
                Role: ND
                Anais da Academia Brasileira de Ciências
                An. Acad. Bras. Ciênc.
                Academia Brasileira de Ciências (Rio de Janeiro )
                March 2000
                : 72
                : 1
                : 83-90
                [1 ] Universidade Federal do Rio de Janeiro Brazil



                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0001-3765&lng=en

                neonatal lupus,auto-immunity,heart blockade,anti-Ro/SSA antibodies


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