Anti-Ro & Anti-La antibodies : Guideline for the management of babies born to mothers with systemic lupus erythematosus (SLE) and other autoimmune disorders

Cord bloods:

FBC and differential WBC

Clinical examination soon after birth – if heart rate > 80 beats per minute and baby clinically stable, he/she should remain with mum.

ECG prior to discharge.
The ECG should be discussed with a neonatal consultant prior to baby’s discharge.

Feeding advice:

Breastfeeding is recommended for most women with SLE as there is no increased risk of neonatal lupus related to breastfeeding. Note that most medications enter breast milk to some extent:

• Diclofenac or ibuprofen can be used, but aspirin should be avoided.
• If NSAIDs are not tolerated, dihydrocodeine may be used with caution as higher doses may lead to drowsiness, or rarely, respiratory depression. 
• Prednisolone can be taken in low doses up to 40 mg/day.
• Chloroquine, hydroxychloroquine and heparin appear to be safe while breastfeeding.
• Azathioprine and cyclosporine enter breast milk in very low concentrations and their continued use can be considered on a case by case basis. This should have been discussed with mother prior to delivery and a plan documented in the handheld records and/or maternal Badger.
• Cyclophosphamide and methotrexate should be avoided during breastfeeding.

Follow up – out patient clinic 2-3 weeks. The family should be advised to contact the neonatal unit for earlier review if baby develops a rash, or if there are any other concerns.

Advice should be given to parents to avoid the baby being exposed to direct sunlight (complete (i.e. Factor 50 or greater) sunblock plus a hat).

Management of babies whose mothers have been treated with TNF inhibitors and other immunosuppressive biological therapy              

(e.g.: Infliximab, Belimumab, Rituximab ) 

Whether infants can receive a live vaccine will depend on the drug involved and how long it was continued for in pregnancy. A plan should be made with an appropriate specialist and the mother during pregnancy. For infants exposed to immunosuppressive biological therapy in utero or through breastfeeding, risk vs benefit of administering a live vaccine should be determined on an individual patient basis. Discuss with pharmacy, an Infectious Diseases specialist or the Public Health team.  The British Society of Rheumatology and the British society of gastroenterology provide the following guidance (which may conflict with manufacturers’ and national guidance):

BSR Guideline on prescribing drugs in pregnancy and breastfeeding: immunomodulatory anti-rheumatic drugs and corticosteroids

In summary:

• Delaying giving live vaccines until 6 months of age is advised for most biologic immunosuppressive agents (Green Book)
• Rotavirus vaccine is safe to give and can be considered after discussion with the parents.
• Manufacturers’ of Infliximab advise avoiding live vaccines until 12 months of age in infants exposed in utero or through breastfeeding unless infant levels are undetectable.
  Consideration should be given to measuring infant levels at 6months of age if there is high need for a live vaccine e.g. BCG before 12 months of age. 

3.1 Congenital heart block

As noted above, the congenital heart block associated with neonatal lupus is considered a form of passively acquired autoimmune disease in which maternal autoantibodies to Ro (SS-A) and La (SS-B) cross the placenta and injure the previously normal fetal heart. While the non-cardiac manifestations of neonatal lupus are generally transient, and resolve with clearance of maternal antibodies from the infant's circulation at several months of age, damage to the conduction system and myocardial disease are irreversible.

Neonatal lupus is usually diagnosed in the presence of a slow heart rate discovered in a fetus or newborn in the absence of associated structural cardiac abnormalities. In utero, the peak onset of the diagnosis of bradycardia is between 18 and 24 weeks of gestation, corresponding to about six weeks after effective placental transport of maternal IgG antibodies begins. The precise mechanism is unknown. The degree of heart block may vary from first degree to third degree block, but most cases diagnosed in utero present with at least second degree or more advanced block. There is a high mortality rate, particularly in fetuses diagnosed with hydrops. Of all cases that are recognised with congenital heart block, the majority will have a pacemaker placed before reaching adulthood.

Babies identified to have a slow heart rate (<80 bpm) should be transferred  to the neonatal unit for a baseline ECG  to confirm the rhythm and a 24 hour ECG recording should be undertaken. The baby should be discussed with paediatric cardiology on-call team prior to transfer to the postnatal ward, to allow outpatient follow up to be arranged. Infants with a mean heart rate < 55 bpm, or any other abnormal cardiac findings should admitted urgently to the NICU, discussed with the on-call neonatology consultant and referred to cardiology as soon as possible. Even with a heart rate of < 55bpm, most neonates will be asymptomatic but if there are signs of low cardiac output, an intravenous infusion of isoprenaline may help to increase the heart rate temporarily before a pacemaker is inserted. Isoprenaline may be sourced from NICU at RHC.

3.2 Pancytopenia

Pancytopenia is rare but isolated thrombocytopenia more common. Most cases will resolve spontaneously over 4 – 6 months with disappearance of maternal antibody; treatment of severe thrombocytopenia or pancytopenia will be symptomatic. Intravenous immunoglobulin may be helpful. Significant abnormalities of the blood count should be discussed with a consultant.

All babies with blood dyscrasia or skin rash should be referred to rheumatology