新生儿红斑狼疮八例分析

Objective Neonatal lupus erythematosus (NLE) is an uncommon" passive autoimmune disease, which is associated with transplacental passage of maternal antibodies. It is often misdiagnosed as intrauterine infection or sepsis. The main purpose of this retrospective study was to summarize its clinical manifestations related with pathogenesis.Methods Data of all the NLE neonates, including clinical manifestations, immunochemical evidence of serum antinuclear antibodies (ANA), antibody to Ro/Sjogren's syndrome A ( anti-Ro/SSA), antibody to La/Sjogren' s syndrome B (anti-La/SSB) and anti-dsDNA antibodies in both infants and mothers, and images from head ultrasound and CT scans were analyzed. Follow-up was performed until one and half years of age or when all the clinical abnormalities had been resolved.Results Totally 8 cases (3 males and 5 females ) seen between September 2003 and February 2006 met the diagnostic criteria of NLE, in whom 4 were small for gestational age and one was born prematurely. Mean gestational age was (38.1 ± 1.9 ) weeks, mean birth weight (2605 ± 420) g, mean admission age (22.4 ± 27.7 ) days (2 hours-72 days) and mean age of onset (9.4 ± 12. 1)days (0-28 days). The common clinical manifestations included cutaneous lupus lesions (8 infants ), neural system abnormalities (2 infants ) and congenital heart block (2 infants). Annular, erythematous or desquamative lesions were seen in skin and all disappeared before 6 months of age. One patient presented with third degree atrio-ventricular block and was delivered by cesarean section because of " fetal distress" He did not recover by the end of one and half years follow-up. One infant was hypotonic with delayed neuro-motor development initially and during follow-up with both abnormal neonatal behavioral neurological assessment (NBNA) and imaging findings. Brain CT scan showed generalized low density involving periventricular and deep white matter at one week of age. At the age of one and a half years, he presented with normal mental development index determined by Child Development Center of China (CDCC) infant intelligence mensuration. Other abnormal clinical findings such as hepatosplenomegaly, anemia, thrombocytopenia, cholestasis and elevated liver enzyme activities were all resolved before 6 months of age. Only 3 mothers of the NLE infants were diagnosed as systemic lupus erythematosus (SLE) before parturition and only one received partial therapy. At least anti-Ro/SSA antibody or anti-La/SSB antibody or ANA was found in the affected patients. Seven cases had circulating anti-Ro and/or anti-La antibodies in the mothers and in the newborns, while ANA was positive in seven newborns and in all mothers. All the clinical symptoms disappeared before 18 months ot age except for congenital heart block. No special intervention was applied.Conclusions Serum auto-antibodies should be investigated to rule out NLE when a newborn infant has congenital heart block or rashes or thrombocytopenia, although there is no maternal history of SLE. Central nervous system abnormalities in NLE are likely to be a transient phenomenon and whether it will cause long-term sequelae is uncertain.