Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity.

OBJECTIVE: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents' and all four grandparents' self-reported ethnicity. The statistical analysis was performed using the Bonferroni's correction (p < 0.0027). RESULTS: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6-18) vs. 12.1(0.3-18) years, p = 0.234], time interval to diagnosis [0.25(0-12) vs. 0.3(0-10) years, p = 0.034], and SLEDAI-2K score [14(0-55) vs. 14(0-63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. CONCLUSIONS: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Key Points • Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. • Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients. • African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia. • The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.

Renal involvement in Henoch-Schönlein purpura: a multivariate analysis of initial prognostic factors.

OBJECTIVES: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schönlein purpura. METHODS: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schönlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis, central nervous system involvement and pulmonary hemorrhage), laboratory tests (serum IgA levels) and treatment given (corticosteroids, intravenous immunoglobulin and immunosuppressive drugs). Patients were divided into two groups (presence or absence of nephritis) and assessed by univariate and multivariate analysis. RESULTS: Evidence of nephritis was detected in 70 patients (49.3%). The univariate analysis revealed that severe abdominal pain (p=0.0049; OR=1.6; 95%CI 1.18-2.21), gastrointestinal bleeding (p=0.004; OR=1.6; 95%CI 1.10-2.26) and corticosteroid use (p=0.0012; OR=1.7; 95%CI 1.28-2.40) were all associated with increased incidence of renal involvement. In the multivariate analysis, logistic regression demonstrated that the only independent variable that predicted nephritis was intense abdominal pain (p<0.012; OR=2.593; 95%CI 1.234-5.452). CONCLUSIONS: Severe abdominal pain was a significant predictor of nephritis in Henoch-Schönlein purpura. Consequently, pediatric patients exhibiting this clinical manifestation should be rigorously monitored, due to the increased risk of renal involvement.

[Chronic recurrent multifocal osteomyelitis of the mandible: report of three cases]. / Osteomielite crônica multifocal recorrente da mandíbula: relato de três casos.

OBJECTIVE: To report three cases of chronic recurrent multifocal osteomyelitis of the mandible, an inflammatory disease affecting one or more bones with absence of isolated microorganisms in affected areas. DESCRIPTION: The first case is a 13 year-old female presenting with pain and fever after dental treatment. The patient received antibiotic treatment for osteomyelitis, but developed progressive enlargement of the mandible and palmoplantar pustulosis. Bone scintigraphy showed intense and diffuse uptake in the mandible. The swelling decreased after indomethacin and hyperbaric oxygen therapy. Case 2 is a 9 year-old female patient with recurrent pain and edema of the right mandible for three years. The diagnosis of osteomyelitis was established and amoxicillin introduced. After three months, tomography showed diffuse mandible osteolysis. Indomethacin and hyperbaric oxygen therapy were introduced, however the patient presented a relapse and was treated with prednisone, rofecoxib and methotrexate. Patient 3, a 10 year-old male, had palmoplantar pustulosis and recurrent enlargement of the mandible. Tomography showed diffuse mandible osteolysis and scintigraphy revealed intense and diffuse uptake in the mandible. The patient was treated with prednisone. Rofecoxib was replaced after two relapses. COMMENTS: Chronic recurrent multifocal osteomyelitis of the mandible is often associated with prolonged pain periods and periods of activity and remission of the inflammatory process. Its recognition is important to prevent the patient from being submitted to prolonged antibiotic therapy and unnecessary invasive procedures.

Envolvimento renal na púrpura de Henoch-Schõnlein: uma análise multivariada de fatores prognósticos iniciais / Renal involvement in Henoch-Schõnlein purpura: a multivariate analysis of initial prognostic factors

OBJETIVOS: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schõnlein. MÉTODOS: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schõnlein admitidos em nosso Hospital Universitário foram revistos. Os fatores preditivos iniciais avaliados nos primeiros 3 meses incluíram: dados demográficos, manifestações clínicas (púrpura palpável persistente, artrite, dor abdominal, dor abdominal intensa, sangramento gastrointestinal, orquite, envolvimento do sistema nervoso central e hemorragia pulmonar), exames laboratoriais (níveis séricos de IgA) e tratamento utilizado (corticosteróides, imunoglobulina endovenosa e medicação imunossupressora). Os pacientes foram divididos em dois grupos (com presença ou ausência de nefrite) e avaliados de acordo com a análise univariada e multivariada. RESULTADOS: Nefrite foi evidenciada em 70 pacientes (49,3 por cento). A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95 por cento 1,18-2,21), sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95 por cento 1,10-2,26) e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95 por cento 1,28-2,40) foram associados com uma maior incidência de envolvimento renal. Na análise multivariada, a regressão logística mostrou que a única variável independente na predição da ocorrência de nefrite foi dor abdominal intensa (p < 0,012; OR = 2,593; IC95 por cento 1,234-5,452). CONCLUSÕES: Dor abdominal intensa representou um preditor significativo da nefrite na púrpura de Henoch-Schõnlein. Conseqüentemente, os pacientes pediátricos com esta manifestação clínica devem ser rigorosamente seguidos, devido ao maior risco de acometimento renal.

OBJECTIVES: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schõnlein purpura. METHODS: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schõnlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis, central nervous system involvement and pulmonary hemorrhage), laboratory tests (serum IgA levels) and treatment given (corticosteroids, intravenous immunoglobulin and immunosuppressive drugs). Patients were divided into two groups (presence or absence of nephritis) and assessed by univariate and multivariate analysis. RESULTS: Evidence of nephritis was detected in 70 patients (49.3 percent). The univariate analysis revealed that severe abdominal pain (p = 0.0049; OR = 1.6; 95 percentCI 1.18-2.21), gastrointestinal bleeding (p = 0.004; OR = 1.6; 95 percentCI 1.10-2.26) and corticosteroid use (p = 0.0012; OR = 1.7; 95 percentCI 1.28-2.40) were all associated with increased incidence of renal involvement. In the multivariate analysis, logistic regression demonstrated that the only independent variable that predicted nephritis was intense abdominal pain (p < 0.012; OR = 2.593; 95 percentCI 1.234-5.452). CONCLUSIONS: Severe abdominal pain was a significant predictor of nephritis in Henoch-Schõnlein purpura. Consequently, pediatric patients exhibiting this clinical manifestation should be rigorously monitored, due to the increased risk of renal involvement.

Osteomielite crônica multifocal recorrente da mandíbula: relato de três casos / Chronic recurrent multifocal osteomyelitis of the mandible: report of three cases

OBJETIVO: Relatar três casos de osteomielite crônica multifocal recorrente de mandíbula. A osteomielite crônica multifocal recorrente é uma doença inflamatória, com acometimento de um ou mais ossos, sem patógenos isolados nas áreas afetadas, sendo o envolvimento da mandíbula raramente descrito na literatura. DESCRIÇÄO: Caso 1 - paciente feminina, com 13 anos, após tratamento dentário evoluiu com dor mandibular e febre. A paciente foi tratada com antibióticos por osteomielite, evoluindo com aumento progressivo da mandíbula e pustulose palmoplantar. A cintilografia óssea mostrou hipercaptaçäo difusa da mandíbula. A reduçäo da tumoraçäo mandibular foi evidenciada com a indometacina e sessöes de câmara hiperbárica. Caso 2 - paciente feminina, com 9 anos, apresentou dor e edema em regiäo de mandíbula direita recorrente por três anos. O diagnóstico de osteomielite foi realizado e introduzido amoxicilina. Após três meses, a tomografia computadorizada mostrou osteólise mandibular difusa. Indometacina e câmara hiperbárica foram introduzidas, porém a paciente apresentou recidiva e foi tratada com prednisona, rofecoxib e metotrexato. Caso 3 - paciente masculino, com 10 anos, apresentou pustulose palmoplantar e aumento difuso da mandíbula recorrente. A tomografia computadorizada de mandíbula evidenciou osteólise mandibular, e a cintilografia óssea, hipercaptaçäo difusa. O paciente foi tratado com prednisona. O rofecoxib foi substituído após duas recidivas. COMENTARIOS: A osteomielite crônica multifocal recorrente da mandíbula pode apresentar um curso doloroso prolongado, com intervalos de atividade e remissäo do processo inflamatório. O seu reconhecimento é importante para evitar uma antibioticoterapia prolongada e procedimentos invasivos desnecessários