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Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus
  1. T L Rivera1,
  2. P M Izmirly1,
  3. B K Birnbaum2,
  4. P Byrne1,
  5. J B Brauth1,
  6. M Katholi1,
  7. M Y Kim3,
  8. J Fischer3,
  9. R M Clancy1,
  10. J P Buyon1
  1. 1
    New York University School of Medicine, New York, USA
  2. 2
    University of Pennsylvania, Philadelphia, Pennsylvania, USA
  3. 3
    Albert Einstein College of Medicine, New York, USA
  1. T L Rivera, Department of Medicine, Division of Rheumatology, New York University School of Medicine, 560 First Avenue, Tisch 407, New York, 10016, USA; tania.rivera{at}nyumc.org

Abstract

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 Sjögren 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.

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Footnotes

  • Competing interests: None declared.

  • Funding: This work was funded by NIH-NIAMS Grant No. NIH, RO1 AR42455-01 (Maternal Autoantibodies: Pathogenesis of Neonatal Lupus) to JPB, NIH Contract NO1-AR-4-2220 (Research Registry for Neonatal Lupus) to JPB, March of Dimes Birth Defects Foundation Grant No. 6-FY05-142 to JPB, AHA Heritage Affiliate Grant No. 0655938T to RMC, and an SLE Lupus Foundation grant to PMI.

  • Ethics approval: Ethics approval was obtained.

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