A combination therapy to treat second-degree anti-Ro/La-related congenital heart block: a strategy to avoid stable third-degree heart block?

Lupus. 2012 May;21(6):666-71. doi: 10.1177/0961203311430969. Epub 2011 Dec 20.

Abstract

While mainly based on the use of fluorinated steroids, there is no standard management of anti-Ro/La-related congenital heart block (CHB). This is a report concerning two consecutive cases of anti-Ro/La-related second-degree block treated with betamethasone (4 mg/day), weekly plasmapheresis, and intravenous immunoglobulins (IVIGs; 1 g/kg) administered every 15 days, a therapy that was begun shortly after CHB was detected and continued until delivery. The newborns were also treated with IVIG (1 g/kg) soon after birth and continued fortnightly until the anti-Ro/La antibody levels became undetectable. In both cases second-degree AV block reverted to a stable sinus rhythm with a first-degree atrioventricular (AV) block. Moreover, there was no recurrence of CHB when therapy was suspended, as confirmed by a 29 month and an eight month follow-up, respectively.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antinuclear / blood*
  • Betamethasone / therapeutic use*
  • Combined Modality Therapy
  • Female
  • Heart Block / blood
  • Heart Block / congenital*
  • Heart Block / immunology
  • Heart Block / therapy
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Infant, Newborn
  • Plasmapheresis*
  • Pregnancy
  • Recurrence
  • Treatment Outcome

Substances

  • Antibodies, Antinuclear
  • Immunoglobulins, Intravenous
  • SS-A antibodies
  • SS-B antibodies
  • Betamethasone

Supplementary concepts

  • Congenital heart block