Update on pediatric systemic lupus erythematosus.
OBJECTIVE
The purpose of this review is to provide an update on the clinical manifestations of SLE in children. Emerging clues on the pathogenesis of the disease based on recent human studies conducted both in children and adults, will also be summarized.


RESULTS
Pediatric Rheumatologists caring for children with SLE face many challenges. As the life expectancy of these patients improves, new recognized complications such as accelerated atherosclerosis and hypertension emerge as major causes of morbidity. However, few longitudinal studies describing the long term outcome of these children, including the impact of disease and treatment on their physical and psychological development are available. Few prospective interventional studies have been carried out to assess the efficacy of established and novel treatments in the pediatric population. Recently, basic studies aimed at understanding the immune alterations underlying this disease have been performed in children. These studies indicate an important role for interferon-alpha (IFN-alpha) in the pathogenesis of this disease and reveal an overall striking homogeneity of leukocyte gene expression profiles in children and adults with SLE. The contribution of novel gene polymorphisms to disease susceptibility and the sequential breakdown of tolerance to nuclear antigens that precedes clinical manifestations in patients with SLE are among the recent studies that are helping us understand the complex SLE puzzle.


CONCLUSIONS
SLE continues to cause significant morbidity in the pediatric age group. A better recognition of the age-specific manifestations and long-term complications of this disease is required to improve its outcome. Understanding its unique pathogenesis will hopefully lead to the development of better, more targeted and less toxic therapies.
Stichweh
Dorothee
D
Baylor Institute for Immunology Research, Dallas, Texas, and the UT Southwestern Medical Center, Dallas, Texas, USA.
Arce
Edsel
E
Pascual
Virginia
V
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D020022:Genetic Predisposition to Disease; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000209:etiology / Q000473:pathology; D010372:Pediatrics / Q000639:trends*; D012219:Rheumatology / Q000639:trends*