RESUMO
OBJECTIVES: To determine the frequency and characteristics of clinical signs, symptoms, laboratory findings, and medication use in children with pediatric systemic lupus erythematosus (pSLE) at presentation and during the course of the disease, and to examine correlations among disease manifestations, disease activity, and damage over time. STUDY DESIGN: The study involved an analysis of medical records and the SLE database of an inception cohort of 256 patients with pSLE (female:male ratio, 4.7:1). RESULTS: The most common clinical manifestations were arthritis (67%), malar rash (66%), nephritis (55%), and central nervous system (CNS) disease (27%). At diagnosis, patients with both renal and CNS disease had the highest SLE Disease Activity Index (SLEDAI) scores (P < .0001), but these scores were similar to those of the total group at 1 year (P = .11). Patients who developed renal and CNS disease more than 1 year after diagnosis had higher SLEDAI scores at disease onset. Some 34% of patients had Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI) scores >1 at a mean follow-up of 3.5 years. A greater proportion of patients with renal and CNS disease had SLICC-DI scores of >1, and these patients had higher mean scores compared with patients without major organ involvement (70% vs 11% [P < .0001] and 1.4 vs 0.1 [P < .0001], respectively). CONCLUSIONS: Most of the patients in our cohort exhibited major organ involvement. These patients had the highest SLEDAI scores at diagnosis, which normalized at 1 year but preceded development of renal and CNS disease. The average SLICC-DI score was lower than that previously reported in patients with pSLE.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Adolescente , Idade de Início , Antimaláricos/uso terapêutico , Autoanticorpos/sangue , Azatioprina/uso terapêutico , Doenças do Sistema Nervoso Central/etiologia , Criança , Pré-Escolar , Progressão da Doença , Uso de Medicamentos/estatística & dados numéricos , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Estimativa de Kaplan-Meier , Nefropatias/etiologia , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Prednisona/uso terapêuticoRESUMO
We describe 3 patients who presented with features of macrophage activation syndrome (MAS) at the time of presentation of systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis, and Kawasaki disease. Immunohistochemical studies in the patient with SLE demonstrated extensive expression of CD163 on hemophagocytic macrophages, suggesting a possible role as a marker of MAS.
Assuntos
Artrite Juvenil/complicações , Lúpus Eritematoso Sistêmico/complicações , Ativação de Macrófagos , Síndrome de Linfonodos Mucocutâneos/complicações , Adolescente , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Artrite Juvenil/imunologia , Artrite Juvenil/metabolismo , Feminino , Humanos , Lactente , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/metabolismo , Masculino , Síndrome de Linfonodos Mucocutâneos/imunologia , Síndrome de Linfonodos Mucocutâneos/metabolismo , Receptores de Superfície Celular/metabolismo , SíndromeRESUMO
To define in children with systemic lupus erythematosus (SLE) the incidence, outcome, and association of thrombocytopenia with other SLE manifestations, specifically thromboembolic events (TEs), we retrospectively reviewed 106 pediatric patients with SLE diagnosed between 1992 and 2001. Thrombocytopenia was found in 50%, which was of a moderate or severe degree in 34%. The thrombocytopenia was sustained in 29% of all patients. Twelve patients were diagnosed with autoimmune thrombocytopenic purpura 2 to 69 months before the diagnosis of SLE. Of them, 10 children required treatment, and three patients underwent splenectomy. TEs occurred in 10.4% of the total cohort of 106. Lupus anticoagulant, but not anticardiolipin antibodies and sustained thrombocytopenia, were associated with a higher risk for TEs.
Assuntos
Lúpus Eritematoso Sistêmico/epidemiologia , Trombocitopenia/epidemiologia , Tromboembolia/epidemiologia , Anticorpos/sangue , Anticorpos/imunologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Prevalência , Taxa de Sobrevida , Trombocitopenia/mortalidade , Tromboembolia/mortalidadeRESUMO
OBJECTIVE: Neonatal lupus erythematosus (NLE) is characterized by complete congenital heart block (CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with autoantibodies directed against SSA/Ro, SSB/La, or both. We carried out a prospective study to determine the incidence of individual NLE features. STUDY DESIGN: The study was performed in two centers: Toronto, Canada, and Milano, Italy. Mothers had been referred for the presence of anti-SSA/Ro autoantibodies, regardless of their diagnosis. All the children were seen at least once within the first 6 months of life for clinical evaluation and laboratory testing. The study group consisted of 128 infants born from 124 pregnancies in 112 women with anti-Ro antibodies with or without anti-La antibodies. RESULTS: There were two cases of CCHB for an overall percentage of 1.6%. Twenty-one children (16%) developed cutaneous NLE. Laboratory testing showed hematologic abnormalities in 27% of the babies and elevation of liver enzymes in 26%. CONCLUSIONS: Mothers with autoimmune diseases and anti-Ro antibodies are at risk of delivering a child with NLE but at a low risk of delivering a child with CCHB. Infants born to mothers with anti-Ro or anti-La antibodies should be monitored for other features of NLE in addition to CCHB.
Assuntos
Autoantígenos/imunologia , Doenças Autoimunes/imunologia , Bloqueio Cardíaco/congênito , Lúpus Eritematoso Sistêmico/epidemiologia , Complicações na Gravidez/imunologia , RNA Citoplasmático Pequeno , Ribonucleoproteínas/imunologia , Adolescente , Adulto , Autoanticorpos/imunologia , Feminino , Bloqueio Cardíaco/imunologia , Humanos , Incidência , Recém-Nascido , Masculino , Gravidez , Estudos ProspectivosRESUMO
OBJECTIVES: To determine if a shorter interval between Kawasaki disease (KD) treatment with intravenous immunoglobulin (IVIG) and fever onset results in increased treatment failures, need for adjunctive therapy, or development of coronary artery lesions. STUDY DESIGN: Patients with KD (n = 178; 89 matched pairs) diagnosed between 1987 and 1999 were included in this case-control study. All patients had fever plus at least 4 of the 5 clinical criteria for KD. Eighty-nine patients who received IVIG at day 5 or earlier were matched to patients diagnosed within 4 weeks and given IVIG at days 6 to 9 of fever. Compiled data from a detailed chart review included demographics, clinical features, fever duration, investigations, disease course, and response to therapy. Differences between matched case and control pairs were analyzed by means oft tests and McNemar tests. RESULTS: No demographic differences were noted between the two groups. Patients treated on day 5 or less of fever had a shorter total fever duration (5.2 +/- 1.9 days vs 8.0 +/- 1.8 days, P <.0001), longer fever after IVIG treatment (1.5 +/- 1.9 days vs 0.8 +/- 1.3 days, P =.008), and less coronary artery ectasia at 1 year after KD onset (4% vs 16%, P =.02). There was no significant difference between cases and control patients in the number of patients with KD recrudescence, need for repeat courses of IVIG, need for corticosteroids, length of hospitalization, or development of coronary artery aneurysms within the first 3 months. Patients who were treated on day 5 or less of fever had higher levels of serum albumin (36 +/- 5 g/L vs 33 +/- 5 g/L, P <.01) and serum ALT (115 +/- 155 U/L vs 46 +/- 49 U/L, P <.001) as well as a lower platelet count (354 +/- 131 vs 403 +/- 166, P =.02) than did control patients during the acute phase. CONCLUSIONS: Early treatment of KD resulted in less coronary ectasia at 1 year after KD onset but was not associated with a quicker resolution of fever, an increased number of treatment failures, an increased need for adjunctive therapy, length of hospitalization, nor development of coronary artery lesions. In children with fever and classic clinical and laboratory findings of KD, treatment with IVIG on or before 5 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.