Long Term Prognosis

Clearly, children with cutaneous NLE do well in the short run. For children with cardiac NLE, there is mortality of ca. 15%-20% (Buyon et al. 1998, Eronen et al. 2000). There is little mortality data concerning hepatobiliary NLE and hematologic NLE.

The finding of 6 deaths in 19 children with hepatobiliary disease in a survey of a national registry indicates that hepatobiliary disease may have a significant mortality rate as well, but more information is needed on this point (Lee et al. 2002).

A long-term concern is the possibility of the development of autoimmune disease later in life. It is to be expected that children with NLE have a slightly increased risk of autoimmunity because by definition they have a family history of autoimmunity. It is the magnitude of the risk that is in question. In a national registry study of 57 children with cutaneous NLE, one child developed Hashimoto's thyroiditis at age 7 years, two developed juvenile rheumatoid arthritis at ages 2 years and 5 years, one developed Raynaud's disease during childhood, and one had a persistently positive high-titer antinuclear antibody (Neiman et al. 2000). This high frequency of autoimmunity at an early age is of concern and points to the advisability of good patient and family education and continued follow-up.

The presence of NLE in a child is an indicator of autoimmunity in the mother. Many mothers are asymptomatic when the child is found to have NLE. With time, however, most mothers develop some symptoms of autoimmunity. Studies differ about which symptoms are most common, with some series indicating Sjogren's symptoms to be most common and others emphasizing LE or undifferentiated connective tissue disease (McCune et al. 1987, Press et al. 1996,Waltuck and Buyon 1994). One group found that mothers of babies with cutaneous NLE were more likely to have symptoms of an autoimmune disorder than were mothers of babies with cardiac NLE (Lawrence et al. 2000).

Mothers who have had one child with NLE are at a relatively high risk for having an affected child in a subsequent pregnancy. Several studies have examined the risk, with results ranging from approximately 1 in 6 pregnancies to 1 in 3 pregnancies resulting in another affected baby (Buyon et al. 1998, Julkunen et al. 1993, McCune et al. 1987, Neiman et al. 2000). It is not unusual for a woman who has had a baby with cutaneous NLE to have a baby with cardiac NLE in a subsequent pregnancy.

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