Abstract

CAGGIANI, MARINA  and  GAZZARA, GUILLERMO. Lupus eritematoso sistémico en niños y adolescentes.: Características clínicas, inmunológicas y evolutivas. Análisis y consideraciones terapéuticas . Arch. Pediatr. Urug. [online]. 2003, vol.74, n.4, pp.237-244. ISSN 1688-1249.

Summary  Systemic lupus erythematosus is an autoimmune disorder on a background of genetic predisposition with chronic evolution. The illness prevalence is low in childhood and adolescence; 20-30% begin in this age. A retrospective analysis of 18 patients followed in a Nephrology Service of the Hospital Pereira Rossell between July 1989 - July 2001 is presented; clinical and immunological features, pathological findings, course and complications. 66% were female, the mean age at diagnosis was 11 years (r: 3y10m-14y11m). 89% presented severe multysistemic involvement. Fever, weakness, malaise and cutaneous involvement were seen in 89%, arthritis in 67%, nephrologic disease in 89%, cardiovascular involvement in 22%, respiratory disease in 22%, central nervous system manifestations in 16,6%. Hematologic abnormalities were seen in 100% of the patients. Antinuclear antibodies were increased in 94,4%, anti ds DNA were positive in 94%, hypocomplementemia was found in 94%. The follow up was of 1 week - 17 years (x: 5y 5m). All the patients received corticosteroids, 9 methylprednisolone bolus. Inmunosupresors were indicated in 16 patients: cyclophosphamide and/or azathioprine. Two patients died (11%), is in remission without treatment one patient, in remission with treatment nine patients, in activity four cases. Two patients were lost of follow up after two and three years; they were in remission under treatment. Infectious diseases were seen in 44%, antiphospholipid syndrome in 28%, bone marrow insufficiency in 22%, gastroduodenits in 16%, glaucoma in 11%. We emphasize the importance of an early diagnosis and adecuate treatment by a multidisciplinary staff to improve the prognosis of this severe illness

Keywords : LUPUS ERYTHEMATOSUS, SYSTEMIC .

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