Neonatal lupus erythematosus P00.8

Aylward, 1928; McCuistion and Schoch, 1954

Rare clinical picture of the fetus, newborn and/or young infant, caused by transferred, specific, antinuclear maternal antibodies. Neonatal lupus erythematosus manifests predominantly with cardiac and/or cutaneous symptoms. The prognosis depends on the presence of a complete AV block, which is associated with a high mortality rate (mortality rate up to 30%).

Panethnic. Incidence: about 1/12,500 live births.

In mothers with a first pregnancy and proven SS-A/-SS-B antibodies, the risk of a newborn infant suffering from neonatal lupus erythematosus is given as 2% (Vanoni F et al. (2017). Studies indicate that the risk is even lower if the mother also has dsDNA antibodies.

Diaplacentally transmitted anti-Ro-(SSA) -antibodies (in > 90% of patients) and anti-La-antibodies (30-40% of patients), more rarely anti-U1-RNP-antibodies, which are considered markers, but also the possibility of genetically determined systemic lupus erythematosus are discussed. The antibodies have a high affinity to the basal keratinocytes and fetal cardiocytes (especially SSA and SSB ak.).

Skin symptoms are congenital or occur during the first weeks of life.

AV block can already be detected intrauterine.

Skin manifestations: Spontaneously reversible, mostly periorally localized, multiple annular plaques reminiscent of subacute cutaneous LE . Also clinical picture of livedo reticularis. There is an increased UV sensitivity (Vanoni F et al. (2017). In addition to the signs of subacute cutaneous lupus erythematosus, transient, slightly atrophic butterfly erythema may also occur without other systemic involvement (Cisse L et al. 2018).

Systemic changes:

• Reversible Coombs-positive anemia
• Intrauterine cardiac involvement with irreversible, incomplete or complete AV block. The occurrence of complete AV block in the fetus is associated with a high mortality rate (mortality rate up to 30%). The irreparable heart damage occurs between the 18th and 24th week of pregnancy. During this time, the maternal antibodies enter the fetal bloodstream through the placenta.
• The known high affinity to the fetal cardiocytes leads to destruction of the AV node, which controls the heartbeat. This can be accompanied by autoimmune-triggered inflammation of the heart muscle and pericardium (fetal myocarditis or perimyocarditis).
• A total AV block is very rare in newborns; the frequency in live-born children is estimated at 1:15,000 to 1:22,000. In 70-90% of cases, neonatal lupus erythematosus is responsible for this disorder. In the majority of cases (>60%), the mothers are completely free of symptoms, so that this pregnancy complication is usually not recognized. Fetal echocardiography is a suitable diagnostic method for checking the cardiac symptoms of the fetus (Sonesson SE et l. 2019).

In 20-70% congenital heart block; passagere thrombocytopenia, cholestatic hepatitis, anemia.

The skin changes usually disappear within 12 months.

AV block: Immediate administration of corticosteroids can improve the child's situation. 50% of newborns with congenital complete AV block require a pacemaker immediately after birth.

In the case of congenital incomplete AV block, careful clinical monitoring is indispensable, as the use of a pacemaker may also become necessary in this constellation in the further course of the disease.

The skin changes will regress within 12 months. The mortality rate for congenital heart block grade 3 is 30% (mainly during the life months 1-3).

As a prophylaxis, the use of dexamethasone can be considered for pregnant women with an increased risk and who have already become pregnant. This should be started by the 17th week of pregnancy. This makes it possible to suppress the fetal autoimmune reaction.

In patients with a desire to have children and high Ro/SS-A or La/SS-B antibodies, the administration of prednisolone (0.5 mg per kg bw/day) over a period of 3 months is recommended before pregnancy. This therapy should be continued at lower doses during pregnancy.

Alternatively: hydroxychloroquine or azathioprine (Dalal DS et al. 2019).

However, the following constellations are generally associated with a risk of developing neonatal lupus erythematosus during pregnancy:

• Pregnant women with a known autoimmune disease (e.g. systemic lupus erythematosus, Sjögren's syndrome, rheumatoid polyarthritis; dermatomyositis)
• High antibody levels of Ro/SS-A or La/SS-B in blood, even without clinical signs of disease
• Previous pregnancies in which the child developed an AV block (10 times higher risk compared to first pregnancy) (Vanoni F et al. (2017)
• Immunogenetic predisposition (detection of HLA-DR3)

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