What Do I Have To Be Worried About With Neonatal Lupus?

Patient Presentation
A 5-year-old male came to clinic for his health supervision visit.
He had moved recently to the area. His mother had no current concerns but said, “I always ask about what I need to be worried about since he had neonatal lupus.”The past medical history revealed that he had neonatal lupus that was diagnosed at birth with a rash.
This resolved within a few weeks and he had no cardiac problems. The patient had otherwise been well.
The family history was positive for diabetes, thyroid disease and rheumatoid arthritis in the maternal family.
Two younger siblings were healthy and did not have neonatal lupus. His mother was also healthy.
The review of systems was normal.
The pertinent physical exam showed he had growth parameters in the 75-95% for age. All his vital signs and physical examination were normal.
The diagnosis of a healthy 5 year old was made.
After the family medicine physician had done the normal anticipatory guidance, she returned to the neonatal lupus question.
She had a computer available and was able to quickly look up a current brief review of neonatal lupus.
The physician was not able to find any information that showed any potential problems for the child who had only cutaneous manifestations of neonatal lupus this far out from birth.
She did tell the mother that she would review information about lupus and other rheumatic diseases, and that they could talk about when the mother came in for her own health supervision visit.

Neonatal lupus erythematosus occurs in 1 of every 20,000 live births, and is caused by passive transfer of maternal antibodies to the fetus.
About 50% of patients with neonatal lupus have mothers who are asymptomatic and the other 50% occurs in mothers with systemic lupus erythematosus or Sjögren syndrome.
In a prospective study of mothers with infants with neonatal lupus, the mothers themselves who were asymptomatic or had an unidentified autoimmune syndrome usually did not progress to have other rheumatic diseases.

Learning Point
The clinical manifestations of neonatal lupus include:

  • Cardiac problems – complete or incomplete congential heart block, but also carditis or myocarditis
  • Skin problems – annular erythematous plaques with a small amount of scale, telangectasias and/or depigmentation
  • Liver problems – hepatospleenomegaly, transaminiemia
  • Platelet problems – thrombocytopenia and petechiae
  • Pulmonary problems – pneumonitis

The antibodies are most commonly anti-SSA/Ro or SSB/La antibodies. Most infants exposed to these antibodies do not develop neonatal lupus.
The risk for an infant to develop neonatal lupus with a mother who tests positive for these antibodies is only 1%. Other factors such as genetics and/or viruses may play a role in which fetuses are affected.
A subsequent child born to a mother who had a child with neonatal lupus, has a 25% risk of having any manifestation of neonatal lupus but most of these manifestations (18-20%) are congenital heart block.
Congenital heart block unfortunately carries a 20% mortality rate and most patients need permanent cardiac pacing.
Luckily, the other abnormalities disappear as the maternal antibodies wane.

Questions for Further Discussion
1. How does neonatal lupus different clinically from systemic lupus erythematosus presenting in childhood?
2. What clinical problems should a neonatologist be ready to treat at birth?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Lupus

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Izmirly PM, Rivera TL, Buyon JP.
Neonatal lupus syndromes.
Rheum Dis Clin North Am. 2007 May;33(2):267-85, vi.

Callen JP. Neonatal Lupus and Cutaneous Lupus Erythematosus in Children. eMedicine.
Available from the Internet at http://www.emedicine.com/ped/topic602.htm (rev. 08/18/2008, cited 10/13/2008).

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

  • Systems Based Practice
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    November 24, 2008