Clinical and pathologic implications of extending the spectrum of maternal autoantibodies reactive with ribonucleoproteins associated with cutaneous and now cardiac neonatal lupus from SSA/Ro and SSB/La to U1RNP.

Abstract:

:While the relationship between maternal connective tissue diseases and neonatal rashes was described in the 1960s and congenital heart block in the 1970s, the "culprit" antibody reactivity to the SSA/Ro-SSB/La ribonucleoprotein complex was not identified until the 1980s. However, studies have shown that approximately 10-15% of cases of congenital heart block are not exposed to anti-SSA/Ro-SSB/La. Whether those cases represent a different disease entity or whether another antibody is associated has yet to be determined. Moreover, the cutaneous manifestations of neonatal lupus have also been identified in infants exposed only to anti-U1RNP antibodies. In this review, we describe what we believe to be the first case of congenital heart block exposed to maternal anti-U1RNP antibodies absent anti-SSA/Ro-SSB/La. The clinical and pathologic characteristics of this fetus are compared to those typically seen associated with SSA/Ro and SSB/La. Current guidelines for fetal surveillance are reviewed and the potential impact conferred by this case is evaluated.

journal_name

Autoimmun Rev

journal_title

Autoimmunity reviews

authors

Izmirly PM,Halushka MK,Rosenberg AZ,Whelton S,Rais-Bahrami K,Nath DS,Parton H,Clancy RM,Rasmussen S,Saxena A,Buyon JP

doi

10.1016/j.autrev.2017.07.013

subject

Has Abstract

pub_date

2017-09-01 00:00:00

pages

980-983

issue

9

eissn

1568-9972

issn

1873-0183

pii

S1568-9972(17)30184-2

journal_volume

16

pub_type

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