Systemic lupus erythematosus (SLE) is an autoimmune disease with varied clinical manifestations. Children and adolescents comprise one-fourth of affected patients with SLE and 40–80% of them have renal involvement. Lupus nephritis (LN) may present with mild urinary abnormalities or fulminant acute nephritis and renal failure. Diffuse proliferative glomerulonephritis (WHO class IV) is the predominant histological presentation in children and more common in boys than girls. This probably is one of the main reasons for the high mortality reported in the initial studies. Early diagnosis and aggressive treatment have led to improvement prognosis in these children. Cytotoxic therapy including intravenous cyclophosphamide has a definite role in the management WHO class IV and occasionally class III lupus nephritis. Prolonged steroid and cytotoxic therapy may lead to significant toxicity.
机构:
NHS Fdn Trust, Paediat Nephrol, Great Ormond St Hosp Children, London WC1N 3JH, EnglandNHS Fdn Trust, Paediat Nephrol, Great Ormond St Hosp Children, London WC1N 3JH, England
Tullus, Kjell
De Mutiis, Chiara
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机构:
Azienda USL, Paediat Unit, Maggiore Hosp, Bologna, ItalyNHS Fdn Trust, Paediat Nephrol, Great Ormond St Hosp Children, London WC1N 3JH, England