We assessed the risk factors associated with death in patients hospitalized for juvenile systemic lupus erythematosus (JSLE) and evaluated the autopsy reports. A total of 57,159 hospitalizations occurred in our institution from 1994 to 2003, 169 of them involving 71 patients with JSLE. The most recent hospitalization of these patients was evaluated. Patients were divided into two groups based on mortality during hospitalization: those who survived (N = 53) and those who died (N = 18). The main causes of hospitalization were JSLE activity associated with infection in 52% and isolated JSLE activity in 44%. Univariate analysis showed that a greater risk of death was due to severe sepsis (OR = 17.8, CI = 4.5-70.9), systemic lupus erythematosus disease activity index (SLEDAI) ≥8 (OR = 7.6, CI = 1.1-53.8), general infections (OR = 6.1, CI = 1.5-25), fungal infections (OR = 5.4, CI = 3.2-9), acute renal failure (OR = 5.1, CI = 2.5-10.4), acute thrombocytopenia (OR = 3.9, CI = 1.9-8.4), and bacterial infections (OR = 2.3, CI = 1.2-7.5). Stratified analysis showed that severe sepsis and SLEDAI ≥8 were not confounder variables. In the multivariate analysis, logistic regression showed that the only independent variable in death prediction was severe sepsis (OR = 98,. Discordance between clinical diagnosis and autopsy was observed in 6/10 cases. Mortality of hospitalized JSLE patients was associated with severe sepsis. Autopsy was important to determine events not detected or doubtful in dead patients and should always be requested.

Objective: to evaluate the hematological involvement (HI) in hospitalized patients with juvenile systemic lupus erythematosus (JSLE). Methods: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria) and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança-University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA) or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl), reticulocytosis, increase in lactate dehydrogenase (LDH) and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. Results: HI occurred in 14 patients (18.9%), with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis). All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. Conclusions: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally associated with an active and systemic disease.

ResumoInfecção pelo vírus varicela zoster (VVZ) em pacientes com lúpus eritematoso sistêmico juvenil (LESJ) tem sido pouco descrita. Durante um período de 12 anos, ocorreram 195 internações em 77 pacientes com LESJ e estas foram acompanhadas pela Unidade de Reumatologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo. Onze pacientes (14%), dez do sexo feminino, apresentaram 14 internações (7,1%) pelo VVZ. Nesses pacientes, a média de idade foi de 16 anos e 5 meses e a média do tempo de duração do LESJ até a primeira infecção devido ao VVZ foi de 4 anos. Todos os episódios das infecções estavam associados com atividade da doença, que se apresentaram como lesões vesicobolhosas seguindo trajeto nervoso. As regiões do tórax e membros foram mais comumente afetadas. Todos haviam utilizado prednisona e quatro usaram ciclofosfamida EV. Todos receberam aciclovir EV por 7 a 10 dias. Nenhum paciente apresentou neuralgia pós-herpética, infecção bacteriana secundária ou evoluiu para óbito. Entretanto, uma paciente em uso de aciclovir apresentou amaurose aguda por vasculite necrosante retiniana bilateral associado ao VVZ, necessitando de duas aplicações de ganciclovir intravítreo e gamaglobulina EV (2 g/kg/dose), com recuperação parcial da acuidade visual. Assim sendo, infecção por VVZ em pacientes com LESJ foi infreqüente, habitualmente associada à atividade da doença e à corticoterapia. Essa infecção foi controlada com aciclovir, e os pacientes raramente apresentaram complicações.Palavras-chave: lúpus eritematoso sistêmico juvenil, corticosteróides, ciclofosfamida, infecção, vírus varicela zoster, aciclovir. AbstRActVaricella zoster virus (VZV) infection in patients with juvenile systemic lupus erythematosus (JSLE) has been rarely described. 195 hospitalizations of 77 JSLE patients occurred in a period of 12 years and were followed at the Pediatric Rheumatology

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