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Systemic Lupus Erythematosus or Aicardi-Goutières Syndrome?

2000; Thieme Medical Publishers (Germany); Volume: 31; Issue: 3 Linguagem: Inglês

10.1055/s-2000-7533

1439-1899

Jean Aicardi, Françoise Goutières,

Inflammasome and immune disorders

In this issue of Neuropediatrics, Dale et al report on a puzzling familial disorder in two brothers born to consanguineous (first cousins) parents, which they consider as infantile systemic lupus erythematosus (SLE). The diagnosis was based on a characteristic autoantibody profile that developed between the ages of 2 and 4 years in children with an early encephalopathic disease with CNS calcification and, in one infant, some evidence of multisystemic involvement. In addition, both infants had, from a few months of age, a rash on the extremities and the older developed nephritis in the course of terminal streptococcal septicemia. The familial character of the disease, its very early onset and the clinical and imaging features clearly differ from those reported in both neonatal and infantile SLE, even though neurological complications have been described in a few such cases [[3], [7]].