Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome.

OBJECTIVE: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. METHODS: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). RESULTS: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1-151) vs. 4 (1-151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5-40) vs. 6 (0-44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. CONCLUSIONS: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.

Sclerotic metaphyseal lines in children and adolescents treated with alendronate.

INTRODUCTION: Bisphosphonates inhibit bone resorption by interfering with the action of osteoclasts. Among the adverse effects, sclerotic lines observed in the metaphysis of long bones have been described as the main imaging finding in pediatric patients. OBJECTIVE: To evaluate the frequency of radiographic changes caused by alendronate in children and adolescents with low bone density or calcinosis. PATIENTS AND METHODS: We conducted a cross-sectional study with 21 patients who were treated with once-weekly alendronate for at least 10 months. Patients underwent x-rays of long bones before the start of alendronate and approximately one year after its use. RESULTS: Eleven patients (52.3%) had sclerotic lines in the metaphysis of long bones. The most frequent site was the tibia (8/11 patients), followed by the femur (7/11), humerus (6/11), radius (4/11), ulna (3/11), and fibula (2/11). Regression of radiographic changes during the study period (up to 1.1 years after discontinuation of alendronate) was not observed. CONCLUSION: If used carefully, alendronate is safe and radiographic changes have not been shown to be clinically relevant.

Linhas escleróticas metafisárias em crianças e adolescentes em uso de alendronato / Sclerotic metaphyseal lines in children and adolescents treated with alendronate

INTRODUÇÃO: Os bisfosfonatos inibem a reabsorção óssea pela interferência na ação dos osteoclastos. Dentre os efeitos adversos, as linhas escleróticas em metáfise de ossos longos são descritas como principal alteração radiográfica na faixa etária pediátrica. OBJETIVO: Avaliar a frequência de alterações radiográficas causadas pelo alendronato utilizado em crianças e adolescentes com baixa densidade óssea ou calcinose. PACIENTES E MÉTODOS: Foi realizado um estudo do tipo coorte retrospectiva analisando-se prontuários de 21 pacientes que fizeram uso de alendronato semanal por no mínimo 10 meses. Os pacientes realizaram radiografias de ossos longos antes do início do alendronato e aproximadamente um ano após o seu uso. RESULTADOS: Onze pacientes (52,3 por cento) apresentaram linhas escleróticas em metáfise dos ossos longos. A localização mais frequente foi em tíbia (8/11 pacientes), seguida de fêmur (7/11), úmero (6/11), rádio (4/11), ulna (3/11) e fíbula (2/11). Nenhum paciente apresentou regressão das alterações radiográficas durante o tempo de evolução (até 1,1 ano após a suspensão do alendronato). CONCLUSÃO: Se usado com critério, o alendronato é seguro e as alterações radiográficas não mostraram ter um significado mais importante.

INTRODUCTION: Bisphosphonates inhibit bone resorption by interfering with the action of osteoclasts. Among the adverse effects, sclerotic lines observed in the metaphysis of long bones have been described as the main imaging finding in pediatric patients. OBJECTIVE: To evaluate the frequency of radiographic changes caused by alendronate in children and adolescents with low bone density or calcinosis. PATIENTS AND METHODS: We conducted a cross-sectional study with 21 patients who were treated with once-weekly alendronate for at least 10 months. Patients underwent x-rays of long bones before the start of alendronate and approximately one year after its use. RESULTS: Eleven patients (52.3 percent) had sclerotic lines in the metaphysis of long bones. The most frequent site was the tibia (8/11 patients), followed by the femur (7/11), humerus (6/11), radius (4/11), ulna (3/11), and fibula (2/11). Regression of radiographic changes during the study period (up to 1.1 years after discontinuation of alendronate) was not observed. CONCLUSION: If used carefully, alendronate is safe and radiographic changes have not been shown to be clinically relevant.

Detection of multifocal osteonecrosis in an adolescent with dermatomyositis using whole-body MRI.

Osteonecrosis is a well-recognized complication of corticosteroid use resulting in significant morbidity, often requiring surgical intervention. Whole-body MRI is a promising method that allows imaging of the whole patient in a reasonable time without the use of ionizing radiation. This technique has the potential for evaluating nonmalignant multifocal skeletal disease like osteonecrosis. This case highlights the value of whole-body MR in an adolescent with dermatomyositis who developed multifocal osteonecrosis.