Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus

T. L. Rivera, P. M. Izmirly, B. K. Birnbaum, P. Byrne, J. B. Brauth, M. Katholi, M. Y. Kim, J. Fischer, R. M. Clancy, J. P. Buyon

Research output: Contribution to journalArticlepeer-review

95 Scopus citations


Objective: To evaluate autoimmune disease progression in asymptomatic and pauci-symptomatic mothers of children with neonatal lupus (NL). Methods: Clinical information on mothers enrolled in the Research Registry for NL (RRNL) was obtained from medical records. Genotyping was performed for -308A/G tumour necrosis factor (TNF)α, 869T/C transforming growth factor (TGF)β and -889C/T interleukin (IL)1α. Results: Of the 321 mothers enrolled, 229 had at least 6 months of follow-up. Of the 51 mothers who were asymptomatic at the NL child's birth, 26 progressed: 12 developed pauci-undifferentiated autoimmune syndrome (pauci-UAS), 2 poly-UAS, 7 SS, 4 SLE and 1 SLE/SS. The median time to develop any symptom was 3.15 years. Of the 37 mothers classified as pauci-UAS at the NL child's birth, 16 progressed: 5 developed poly-UAS, 6 Sjö0gren syndrome (SS), 4 systemic lupus erythematosus (SLE) and 1 SLE/SS. Of the pauci-UAS mothers enrolled within 1 year, the median time to progression was 6.7 years. Four mothers developed lupus nephritis (two asymptomatic, two pauci-UAS). The probability of an asymptomatic mother developing SLE by 10 years was 18.6%, and developing probable/definite SS was 27.9%. NL manifestations did not predict disease progression in an asymptomatic mother. Mothers with anti-Sjögren syndrome A antigen (SSA/)Ro and anti-Sjögren syndrome B antigen (SSB)/La were nearly twice as likely to develop an autoimmune disease as mothers with anti-SSA/Ro only. Only TGFβT/T was significantly higher in SLE mothers compared to asymptomatic mothers (p = 0.03). Conclusions: Continued follow-up of asymptomatic NL mothers is warranted since nearly half progress, albeit few develop SLE. While the anti-SSB/La antibodies may be a risk factor for progression, further work is needed to determine reliable biomarkers in otherwise healthy women with anti-SSA/Ro antibodies identified solely because of an NL child.

Original languageEnglish (US)
Pages (from-to)828-835
Number of pages8
JournalAnnals of the Rheumatic Diseases
Issue number6
StatePublished - Jun 2009

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)


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