NEONATAL LUPUS SYNDROMES - 11/09/11
Résumé |
Nearly a century ago, Morquio first described the rare defect of isolated congenital heart block (CHB).86 In 1928, Aylward reported the occurrence of CHB in two children whose mother “suffered from Mikulicz's disease.”4 By the 1970s, this curious clinical observation was further solidified with reports of many children with CHB whose mothers had autoimmune diseases31, 82, 121 and by the finding that the maternal sera contained antibodies to SSA/Ro and SSB/La ribonucleoproteins.71, 101 Indeed, CHB is currently considered a model of passively acquired autoimmunity, whereby immune abnormalities in the mother lead to the production of autoantibodies that cross the placenta and presumably injure the otherwise normally developing fetus. Other neonatal abnormalities affecting the skin,117 liver,67 and blood elements were also reported to be associated with anti-SSA/Ro-SSB/La antibodies in the maternal and fetal circulation and are now grouped under the heading of neonatal lupus syndromes. Neonatal lupus was so termed because the cutaneous lesions of the neonate resembled those seen in systemic lupus erythematosus (SLE).65, 83 To date, CHB is irreversible and most commonly detected during the second trimester.116 In contrast, the noncardiac manifestations of neonatal lupus are transient, resolving at about 6 months of life, coincident with the disappearance of maternal autoantibodies from the neonatal circulation (Table 1).
Despite their rarity, the so-called neonatal lupus syndromes attract considerable attention, probably because maternal autoantibodies in this disease may well be pathogenic and fetal disease therefore preventable. Many perplexing issues remain unexplained, such as the spectrum of disease manifestations: some infants have only cardiac manifestations, some skin, and others both (even with the same mother). Why is the fetal conduction system targeted, and not the maternal cardiac system? In addition, the mechanism of tissue injury mediated by antibodies reactive with intracellular SSA/Ro-SSB/La polypeptides has yet to be defined. Clinically, the neonatal lupus syndromes present a unique challenge to the team of rheumatologist, obstetrician, perinatologist, neonatologist, and pediatric cardiologist: to identify the pregnancy at risk for disease development, to effect its timely detection, and to devise in utero and postnatal treatment strategies. Recently, there have been important advances on several fronts, both at the bench and at the bedside. This review covers histopathology, SSA/Ro-SSB/La antigen-antibody systems, immunogenetics, clinical manifestations, diagnosis, and management strategies.
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| Address reprint requests to Jill P. Buyon, MD, Department of Rheumatology, Room 1608, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003 This work was supported in part by grants to Dr. Buyon from the National Institutes of Health (AR42455-01), and American Heart Association, New York Affiliate, and a fellowship to Dr. Tseng from the Arthritis Foundation, New York Chapter. The Research Registry for Neonatal Lupus (NIH-NIAMS Contract NO1 AR-4-2220) has provided information on a large number of patients. |
Vol 23 - N° 1
P. 31-54 - février 1997 Retour au numéro
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- SYSTEMIC LUPUS ERYTHEMATOSUS FLARES DURING PREGNANCY
- Munther A. Khamashta, Guillermo Ruiz-Irastorza, Graham R.V. Hughes
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- Anne Lynch, Robert Silver, Woodruff Emlen
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