Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study.

BACKGROUND: Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations. PROCEDURES: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE. ES was assessed at disease diagnosis and defined by the combination of immune thrombocytopenia and autoimmune hemolytic anemia. RESULTS: ES was observed in 11 of 850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (91%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multiorgan involvement, autoantibodies profile, and low complement (P > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, P = 0.003) and musculoskeletal involvement (18% vs. 69%, P = 0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, P = 0.013) and intravenous immunoglobulin use (64% vs. 3%, P < 0.0001) were significantly higher in the ES group, with similar current prednisone dose between groups (1.1 [0.76-1.5] vs. 1.0 mg/kg/day [0-30], P = 0.195). CONCLUSIONS: Our large multicenter study identified ES as a rare and severe initial manifestation of active cSLE with good outcome. Diagnosis is challenging due to the lack of typical signs and symptoms of lupus and the requirement to exclude infection and primary immunodeficiency.

High frequency of calcinosis in juvenile dermatomyositis: a risk factor study.

OBJECTIVE: To assess the frequency of calcinosis in patients with juvenile dermatomyositis, and the possible risk factors for that manifestation. METHODS: Medical record review of 34 patients, with an emphasis on the following characteristics: demographic, clinical and laboratory data; type of treatment; adherence to treatment; disease course (monocyclic, chronic and polycyclic); and disease severity. Patients were divided into two groups as follows: those who developed calcinosis (up to the sixth month of follow-up and after six months of follow-up) and those who did not develop calcinosis. Twenty-seven patients underwent two nailfold capillaroscopies (NFC), which were considered altered when the scleroderma pattern was found. RESULTS: The mean age of symptom onset of the 34 patients was 6.5 years, the time until diagnosis was 1.2 years, and 70% were females. Half of the patients had a monocyclic disease course, and only 14.7% had severe vasculitis. Almost 90% of the patients undergoing NFC showed a change on the first assessment, 74% showed a change on the second assessment, and the mean interval between both assessments was 1.6 year. Calcinosis was evidenced in 16 (47.1%) patients. No association was observed between the variables analyzed and the development of calcinosis. CONCLUSION: No risk factors for calcinosis were identified in this study, although that complication was found in half of the patients with juvenile dermatomyositis studied.

Frequência elevada de calcinose em dermatomiosite juvenil: estudo de fatores de risco / High frequency of calcinosis in juvenile dermatomyositis: a risk factor study

OBJETIVO: Avaliar a frequência de calcinose em pacientes com dermatomiosite juvenil, bem como estudar possíveis fatores de risco para essa manifestação. MÉTODOS: Revisão de prontuários de 34 pacientes, com ênfase nas características demográficas, clínicas e laboratoriais, tipo de tratamento e adesão, tipo de evolução (monocíclico, crônico e policíclico) e gravidade da doença. Os pacientes foram separados em grupos: aqueles que desenvolveram calcinose (até o sexto mês de acompanhamento ambulatorial e após seis meses de acompanhamento) e os que não desenvolveram calcinose. Vinte e sete pacientes fizeram dois exames de capilaroscopia periungueal (CPU), os quais foram considerados alterados quando era encontrado padrão escleroderma. RESULTADOS: A média de idade de início dos sintomas dos 34 pacientes foi de 6,5 anos, e o tempo até o diagnóstico foi de 1,2 anos. Setenta por cento eram meninas. Metade dos pacientes teve curso monocíclico da doença, e apenas 14,7% tiveram vasculite grave. Quase 90% dos pacientes que realizaram CPU tiveram alteração na primeira avaliação, e 74% tiveram alteração na segunda avaliação, com uma média de 1,6 anos entre as duas. Dezesseis (47,1%) pacientes apresentaram calcinose. Não houve associação entre as variáveis analisadas e o desenvolvimento da calcinose. CONCLUSÃO: Não conseguimos demonstrar a presença de fatores de risco para calcinose, apesar de termos encontrado uma frequência dessa complicação em cerca de metade dos pacientes com dermatomiosite juvenil.

OBJECTIVE: To assess the frequency of calcinosis in patients with juvenile dermatomyositis, and the possible risk factors for that manifestation. METHODS: Medical record review of 34 patients, with an emphasis on the following characteristics: demographic, clinical and laboratory data; type of treatment; adherence to treatment; disease course (monocyclic, chronic and polycyclic); and disease severity. Patients were divided into two groups as follows: those who developed calcinosis (up to the sixth month of follow-up and after six months of follow-up) and those who did not develop calcinosis. Twentyseven patients underwent two nailfold capillaroscopies (NFC), which were considered altered when the scleroderma pattern was found. RESULTS: The mean age of symptom onset of the 34 patients was 6.5 years, the time until diagnosis was 1.2 years, and 70% were females. Half of the patients had a monocyclic disease course, and only 14.7% had severe vasculitis. Almost 90% of the patients undergoing NFC showed a change on the first assessment, 74% showed a change on the second assessment, and the mean interval between both assessments was 1.6 year. Calcinosis was evidenced in 16 (47.1%) patients. No association was observed between the variables analyzed and the development of calcinosis. CONCLUSION: No risk factors for calcinosis were identified in this study, although that complication was found in half of the patients with juvenile dermatomyositis studied.