[Unfavorable outcomes in disseminated lupus erythematosus in children. Cooperative study in the Paris region].
Pediatric cases of systemic lupus erythematosus with an unfavorable outcome (terminal renal failure requiring chronic hemodialysis, or death) assembled during a retrospective multicenter study of pediatric SLE in the Paris metropolitan area were analyzed. Seven patients (6 girls, 1 boy) were entered into a chronic hemodialysis program. Four had diffuse proliferative glomerulonephritis, the pattern of glomerular disease classically responsible for end-stage renal failure. The other three patients had membranous glomerulonephritis with active segmental lesions, a form of glomerulopathy whose severe prognosis deserves to be emphasized. Nine other patients (8 girls, 1 boy) died. In six patients, death occurred as a result of a flare with malignant hypertension and progressive renal failure (1 case), pancreatitis (1 case), encephalopathy (2 cases) or cardiomyopathy (2 cases). An infectious disease (tuberculosis, mumps) was apparently the cause of the two cases of encephalopathy. One girl died as a result of a hemorrhagic syndrome with a cerebral hematoma. Two other girls died at home. Overall, among 111 children with SLE 14% had an unfavorable outcome. Sex and age at onset seemed to have no bearing on prognosis. Patients with renal involvement were apparently more likely to have an unfavorable outcome. Lastly, although the influence of ethnic origin is unproven, children living in foreign countries of French overseas territories, but treated in France have an increased risk for unfavorable outcomes.
Unité 155 INSERM, Château de Longchamp, Paris.
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D000293:Adolescent; D001706:Biopsy; D002648:Child; D005260:Female; D006801:Humans; D008181:Lupus Nephritis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D008297:Male; D017063:Outcome Assessment, Health Care; D010297:Paris / Q000453:epidemiology; D011379:Prognosis; D006435:Renal Dialysis / Q000592:standards*; D015996:Survival Rate
Les évolutions défavorables au cours du lupus érythémateux disséminé de l'enfant. Etude coopérative en région parisienne.