Rheumatology Neonatal

Basic & Clinical Research

Title: Neonatal Lupus National Research Registry

Investigator: Jill P. Buyon, M.D.

Funding: NIH - NIAMS

Dates: 1994 - 1999

Summary: Congenital heart block (CHB), detected at or before birth in a structurally normal heart, is strongly associated with autoantibodies reactive with 48kD SSB/La, 52kD SSA/Ro and 60kD SSA/Ro proteins. Information on the proportion of children requiring pacemakers, mortality, and recurrence rates in a subsequent pregnancy is critical for family counseling, research strategies and management. The National Research Registry for Neonatal Lupus was initiated in September 1994 to establish a database of mothers whose children have complete congenital heart block (CHB) and/or other manifestations (dermatologic, hepatic, hematologic) of neonatal lupus. As of 1/15/99, 211 mothers of 247 affected children have been enrolled, and 27 research protocols have been approved and are currently active. We welcome physician referrals of eligible patients, and encourage interested researchers to submit protocols for acquisition from the Registry of data, sera and/or subjects willing to participate in research.

PUBLICATIONS:

1. Buyon JP, Winchester RJ. Complete congenital heart block: a model of passively acquired autoimmunity. Arthritis Rheum 1990; 33:609-614.

2. Buyon JP, Winchester RJ, Slade SG, Arnett, F, Copel J, Friedman D, Lockshin MD. Identification of mothers at risk for congenital heart block and other neonatal lupus syndromes in their children: Comparison of ELISA and immunoblot to measure anti-SSA/Ro and anti-SSB/La antibodies. Arthritis Rheum 1993; 36:1263-1273.

3. Waltuck J, Buyon JP. Autoantibody associated complete heart block: Outcome in mothers and children. Annals Internal Med 1994; 120:544-551

4. Brucato A, Franceschini F, Buyon JP. Neonatal lupus: Long term outcomes of mothers and children and recurrence rate. Clinical & Experimental Rheumatology 1997; 15:467473.

5. Buyon JP. Autoantibodies reactive with SSA/Ro-SSB/La and pregnancy. VIth International Symposium on Sjögren's Syndrome, University of Connecticut, October 15-18, 1997. J Rheum 1997; 24(Suppl 50).

6. Tseng C, Friedman D, Buyon JP: Spectrum of cardiac histopathology in cases of autoimmune-associated congenital heart block (CHB) obtained from the Research Registry for Neonatal Lupus [abstract]. Arthritis Rheum. 1997; 40 (Suppl.):S333.

7. Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, Lee L, Marston K, Provost T, Reichlin M, Rider L, Rupel A, Saleeb S, Weston W, Skovron ML. Autoimmune-associated congenital heart block: Mortality, morbidity, and recurrence rates obtained from a national neonatal lupus registry. J. Am. Coll. Cardiology 1998; 31:1658-1666.

8. Boutjdir M, Chen L, Zhang ZH, Tseng CE, El-Sherif N, Buyon JP. Serum and IgG from the mother of a child with congenital heart block induce conduction abnormalities and inhibit L-type calcium channels in a rat heart model. Ped. Research 1998; 44:11-19.

9. Miranda-Carus ME, Tseng CE, Rashbaum W, Ochs RL, Casiano CA, DiDonato F, Chan EKL, Buyon JP. Accessibility of SSA/Ro and SSB/La antigens to maternal autoantibodies in apoptotic human fetal cardiac myocytes. J. Immunol. 1998; 161:5061-6059.

10. Buyon JP. Neonatal lupus syndrome. In Systemic Lupus Erythematosus, 3rd Ed. Lahita R, ed. Academic Press. 1998; pp. 337-359.

11. Buyon JP, Brucato A. Neonatal lupus. Seminars in Clinical Immunology 1998; 15:5-19.

12. Saleeb S, Copel J, Friedman D, Buyon JP. Effect of fluorinated glucocorticoids on outcome of autoimmune-associated congenital heart block (abstract). Arthritis Rheum. 1998; 41(suppl):S80.

13. Abbot AE, Buyon JP, Friedman D. The prevalence of cardiomyopathy observed in patients recorded in the Research Registry for Neonatal Lupus. Arthritis Rheum. 1998; 41(suppl):S262.

14. Tseng CE, Miranda E, DiDonato F, Boutjdir M, Rashbaum W, Chan EKL, Buyon JP. mRNA and protein expression of SSA/Ro and SSB/La in human fetal cardiac myocytes cultured using a novel application of the Langendorff procedure. Ped. Research (in press, 1999).

15. Buyon JP. The role of anti-SSA/Ro-SSB/La autoantibodies in neonatal lupus. In Lupus: Cellular and Molecular Pathogenesis. Kammer GM, Tsokos GC, eds. Humana Press (in press, 1998).

16. Saleeb S, Copel J, Friedman D, Buyon JP. Comparison of treatment with fluorinated glucocorticoids to natural history of autoantibodyassociated congenital heart block: Retrospective review of the Research Registry for Neonatal Lupus. Submitted.

17. Moak JP, Barron KS, Hougen TJ, Cohen MH, Nordenberg A, Van Hare GF, Friedman RA, Perez M, Cecchin F, Schneider DS, Nehgme RA, Buyon JP. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. (Submitted for publication).

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