Neonatal lupus erythematosus: Difference between revisions
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The rash of neonatal lupus generally does not cause scarring and disappears within six to eight months. Appearance of skin lesions postnatally is independent of breastfeeding. Thus, breastfeeding is not contraindicated in mothers with anti-SSA/Ro and/or anti-SSB/La antibodies.<ref name="pmid19578103">{{cite journal| author=Klauninger R, Skog A, Horvath L, Winqvist O, Edner A, Bremme K et al.| title=Serologic follow-up of children born to mothers with Ro/SSA autoantibodies. | journal=Lupus | year= 2009 | volume= 18 | issue= 9 | pages= 792-8 | pmid=19578103 | doi=10.1177/0961203309103188 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19578103 }} </ref> Infants and young children with complete heart block who are asymptomatic usually remain well until later childhood, adolescence, or adulthood. However, exercise limitation and even death are possible in the absence of pacing. The prognosis following pacemaker implantation is excellent for most children, although development of heart failure may occur. | The rash of neonatal lupus generally does not cause scarring and disappears within six to eight months. Appearance of skin lesions postnatally is independent of breastfeeding. Thus, breastfeeding is not contraindicated in mothers with anti-SSA/Ro and/or anti-SSB/La antibodies.<ref name="pmid19578103">{{cite journal| author=Klauninger R, Skog A, Horvath L, Winqvist O, Edner A, Bremme K et al.| title=Serologic follow-up of children born to mothers with Ro/SSA autoantibodies. | journal=Lupus | year= 2009 | volume= 18 | issue= 9 | pages= 792-8 | pmid=19578103 | doi=10.1177/0961203309103188 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19578103 }} </ref> Infants and young children with complete heart block who are asymptomatic usually remain well until later childhood, adolescence, or adulthood. However, exercise limitation and even death are possible in the absence of pacing. The prognosis following pacemaker implantation is excellent for most children, although development of heart failure may occur. | ||
== Diagnosis == | ==Diagnosis== | ||
Women who test positive for SSA/Ro and SSB/La autoantibodies may benefit from more intensive assessment for fetal heart block with frequent fetal echocardiographic testing during pregnancy. Complete heart block (and usually second degree block) results in fetal bradycardia that can be detected by routine fetal auscultation, ultrasonography, or echocardiography. | |||
== Treatment == | == Treatment == | ||
Revision as of 19:49, 26 August 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:; NL; NLE; NLS, neonatal lupus; neonatal lupus syndrome
Overview
Neonatal lupus is a passively transferred autoimmune disease. It occurs in about 1 to 2 percent of babies born to mothers with autoimmune disease, primarily systemic lupus erythematosus (SLE) and Sjögren’s syndrome, and antibodies to SSA/Ro and/or SSB/La. The most serious complication of neonatal lupus is complete heart block.
Historical Perspective
The first case reported by Aylward in 1928, who described two siblings with complete heart block born to a mother who had Sjogren’s syndrome. Plant & Stevens described complete heart block as a manifestation of neonatal lupus in 1945.[1] But the first report linking autoimmune disease in mother with cutaneous lupus was McCuistion and Schoch in 1954. In 1957 Hogg noted the possible relation between autoimmune disease of the mother and congenital heart block in her child. Finally in 1980 Weston reported the association of neonatal lupus with maternal anti-Ro auto- antibodies.[2]
Pathophysiology
Neonatal lupus is presumed to result from transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Ro and La molecules are thought to form a single particle that is present in all cells. The precise mechanism of injury to specific tissues, such as the skin and heart, is not known. The auto antibodies are usually anti-SSA/Ro against usually 52kD or 60kD protein or anti-SSB/La against 48kD protein These auto antibodies later enter the myocardial cell causing exaggerated apoptosis which leads to expression of the antibodies on the surface of the cardiocyte. The same maternal antibodies recognize the antigens present in the neonatal skin exposed to UV light and high estradiol concentrations and cause the cutaneous manifestations of neonatal lupus.
Genetics
Genetic factors in the infant, in particular the HLA alleles DQB102, DRB103, and a polymorphism in the promoter region of the gene for tumor necrosis factor alpha (-308A, associated with higher TNFa production) may play a role in skin disease.
Differentiating Neonatal Lupus Erythematosus from other Diseases
The causes of isolated annular erythematous lesions include erythema annulare centrifugum, erythema marginatum, tinea corporis, seborrheic dermatitis and ichthyosiform genodermatosis. In patients without neonatal lupus or significant congenital structural heart disease, familial inheritance of conduction disease is often responsible for AV block.
Epidemiology and Demographics
The prevalence of Anti-SSA antibodies in women is 1:200 while the incidence of neonatal lupus is only 1 in 20,000 live births.[3] Only 1-2% of the infants of mothers with anti-SSA/Ro with or without anti-SSB/La antibodies develop neonatal lupus, although it ranges from 0.6% to 25% with an average of 7.2% by various studies. The incidence increases to 3% if the mother has anti- La antibodies in addition to anti- Ra antibodies. If the mother has also SLE along with anti-SSA antibodies the incidence of NLE may be up to 6-13%. This is much higher and reaches up to 25% if the mother already had a child with neonatal lupus. 15-20% present as complete heart block and 6% present as cutaneous lupus. The incidence of CHB is seen in 50-60% while the cutaneous lupus is seen in 25-30% and the combination of cutaneous and cardiac manifestations seen only in 4-10% of the patients with neonatal lupus.[4]
Risk Factors
Presence of maternal antibodies to SSA/Ro and/or SSB/La is a powerful risk factor for congenital heart block, however it is not the only determinant of the development of neonatal lupus. Maternal antibodies to other antigens may cause neonatal disease in some cases. As an example, anti-U1 RNP antibodies in the absence of anti-SSA/Ro or anti-SSB/La antibodies were found in a few instances.[5][6] These patients had the classic rash of NL, but not congenital heart block. There is one report of cardiac injury associated with anti-RNP absent anti-SSA/Ro-SSB/La, and that was transient first degree block.[7] The fact that heart block develops in only a minority of subsequent pregnancies, despite the persistence of maternal anti-SSA/Ro and/or anti-SSB/La antibodies, strongly suggests that fetal factors are important determinants of risk. Genetic factors in the infant, in particular the HLA alleles DQB102, DRB103, and a polymorphism in the promoter region of the gene for tumor necrosis factor alpha (-308A, associated with higher TNFa production) may play a role in skin disease.
Screening
Prenatal screening for anti-SSA/Ro and anti-SSB/La antibodies is warranted for women who are known to be at risk. Women who are more likely to have anti-SSA/Ro and anti-SSB/La antibodies include those with lupus, Sjögren syndrome, an undifferentiated autoimmune disease, or having a baby with neonatal lupus in a previous pregnancy.
Natural History, Complications and Prognosis
The rash of neonatal lupus generally does not cause scarring and disappears within six to eight months. Appearance of skin lesions postnatally is independent of breastfeeding. Thus, breastfeeding is not contraindicated in mothers with anti-SSA/Ro and/or anti-SSB/La antibodies.[8] Infants and young children with complete heart block who are asymptomatic usually remain well until later childhood, adolescence, or adulthood. However, exercise limitation and even death are possible in the absence of pacing. The prognosis following pacemaker implantation is excellent for most children, although development of heart failure may occur.
Diagnosis
Women who test positive for SSA/Ro and SSB/La autoantibodies may benefit from more intensive assessment for fetal heart block with frequent fetal echocardiographic testing during pregnancy. Complete heart block (and usually second degree block) results in fetal bradycardia that can be detected by routine fetal auscultation, ultrasonography, or echocardiography.
Treatment
References
- ↑ PLANT RK, STEVEN RA (1945). "Complete A-V block in a fetus, case report". Am Heart J. 30: 615–8. PMID 21008284.
- ↑ Lee LA, Weston WL (1997). "Cutaneous lupus erythematosus during the neonatal and childhood periods". Lupus. 6 (2): 132–8. PMID 9061661.
- ↑ Neiman AR, Lee LA, Weston WL, Buyon JP (2000). "Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry". J Pediatr. 137 (5): 674–80. doi:10.1067/mpd.2000.109108. PMID 11060534.
- ↑ Eronen M, Sirèn MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T (2000). "Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn". Pediatrics. 106 (1 Pt 1): 86–91. PMID 10878154.
- ↑ Provost TT, Watson R, Gammon WR, Radowsky M, Harley JB, Reichlin M (1987). "The neonatal lupus syndrome associated with U1RNP (nRNP) antibodies". N Engl J Med. 316 (18): 1135–8. doi:10.1056/NEJM198704303161807. PMID 3494943.
- ↑ Sheth AP, Esterly NB, Ratoosh SL, Smith JP, Hebert AA, Silverman E (1995). "U1RNP positive neonatal lupus erythematosus: association with anti-La antibodies?". Br J Dermatol. 132 (4): 520–6. PMID 7748740.
- ↑ Acherman RJ, Friedman DM, Buyon JP, Schwartz J, Castillo WJ, Rollins RC; et al. (2010). "Doppler fetal mechanical PR interval prolongation with positive maternal anti-RNP but negative SSA/Ro and SSB/La auto-antibodies". Prenat Diagn. 30 (8): 797–9. doi:10.1002/pd.2544. PMID 20582918.
- ↑ Klauninger R, Skog A, Horvath L, Winqvist O, Edner A, Bremme K; et al. (2009). "Serologic follow-up of children born to mothers with Ro/SSA autoantibodies". Lupus. 18 (9): 792–8. doi:10.1177/0961203309103188. PMID 19578103.