Rheumatology International

, Volume 26, Issue 10, pp 886–895

Identification and management of fetuses at risk for, or affected by, congenital heart block associated with autoantibodies to SSA (Ro), SSB (La), or an HsEg5-like autoantigen

  • Renate Claus
  • Heiko Hickstein
  • Thomas Külz
  • Ute Lenschow
  • Doris Meiske
  • Andrea Kotitschke
  • Hans-Jürgen Thiesen
  • Peter Lorenz
Original Article

DOI: 10.1007/s00296-005-0101-4

Cite this article as:
Claus, R., Hickstein, H., Külz, T. et al. Rheumatol Int (2006) 26: 886. doi:10.1007/s00296-005-0101-4

Abstract

The congenital heart block (CHB), diagnosed in structurally normal hearts, is strongly associated with, if not caused by, maternal SSA/SSB antibodies (Abs). It develops between 16 and 24 weeks’ gestation, coincidentally with the increased transplacental IgG passage, and a window of unique cardiac vulnerability. Less is known about rare CHB cases in which neither cardiac malformations nor SSA/SSB Abs are detectable. We report on four pregnant women: patient 1 at high CHB risk (owing to Sjögren’s syndrome (SS) and recurrent pregnancy losses), and patients 2–4 with already established CHB (aggravated by hydrops in patient 2). Abs were found directed to SSA/SSB (patients 1–3) or to an HsEg5-like autoantigen instead (patient 4). During preventive immunoadsorption (IA) from week 19 throughout (patient 1), or therapeutic IA (plus dexamethasone), commenced at week 25 (patient 2), SSA Ab levels decreased per session by 47±7 or 80±16%, respectively, and hydropic changes resolved. Patient 1 delivered a healthy boy, while patients 2–4 gave birth to CHB-affected children at need for permanent pacing. The irreversibility of complete CHB may justify (a) early ANA screening in all pregnancies (thereby also considering specificities as anti-HsEg5), and (b) preventive immmunoadsorption in high-risk pregnancies (before/during the critical cardiac development phase). This implies controversy, because factors converting risk to disease (in only ~2%) are unknown, and prospective randomized treatment studies are not available, given the rarity of CHB.

Keywords

Congenital heart blockAutoantibodiesSSA (Ro)SSB (La)HsEg5 (NuMA-2)Plasmapheresis-immunoadsorption

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Renate Claus
    • 1
  • Heiko Hickstein
    • 2
  • Thomas Külz
    • 3
  • Ute Lenschow
    • 4
  • Doris Meiske
    • 1
  • Andrea Kotitschke
    • 1
  • Hans-Jürgen Thiesen
    • 1
  • Peter Lorenz
    • 1
  1. 1.Department of Immunology, Medical FacultyUniversity of RostockRostockGermany
  2. 2.Department of Internal Medicine, Medical FacultyUniversity of RostockRostockGermany
  3. 3.Department of Obstetrics and Gynecology, Medical FacultyUniversity of RostockRostockGermany
  4. 4.Department of Pediatrics, Medical FacultyUniversity of RostockRostockGermany