Childhood-Onset Systemic Lupus Erythematosus (cSLE): An International Perspective.

PURPOSE OF REVIEW: Childhood-onset systemic lupus erythematosus (cSLE) is a severe and potentially life-threatening chronic autoimmune disease. cSLE is more aggressive and has poorer outcomes than adult-onset disease. The global burden of cSLE is poorly understood, with most publications on cSLE originating from high-resourced settings. The reports from less resourced settings indicate high morbidity and mortality in these populations. RECENT FINDINGS: In this article, we review the disparities in global access to rheumatology care and research for patients with cSLE. We highlight recent cSLE advances from all regions of the globe. We describe current obstacles to cSLE clinical care and research in all settings. Finally, we propose a path forward for high quality, equitable and accessible care to individuals with cSLE everywhere. Individuals with cSLE are at risk for morbidity and death, yet patients worldwide face challenges to adequate access to care and research. Sustained, collaborative efforts are needed to create pathways to improve care and outcomes for these patients.

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.

Remission status follow-up in children with juvenile idiopathic arthritis

OBJETIVO: Caracterizar a atividade inflamatória articular e sistêmica na artrite idiopática juvenil (AIJ), determinando o estado de remissão com e sem uso de medicação. MÉTODOS: Um total de 165 casos de AIJ, acompanhados em média por 3,6 anos, foram revisados para caracterização de episódios de inatividade, remissão clínica com e sem medicação. Os dados obtidos foram analisados por meio de estatística descritiva, análise de sobrevida, comparação das curvas de Kaplan-Meier, teste de log rank e análise de regressão logística binária para determinação de fatores preditivos para a remissão ou atividade persistente. RESULTADOS: Dos casos revisados, 108 preencheram os critérios de inclusão: 57 pacientes (52,7 por cento) apresentaram um total de 71 episódios de inatividade, com 2,9 anos em média para cada episódio; 36 episódios (50,7 por cento) de inatividade resultaram em remissão clínica sem medicação, sendo 35 por cento do subtipo oligoarticular persistente. A probabilidade de remissão clínica com medicação em 2 anos foi de 81, 82, 97 e 83 por cento para casos de AIJ oligoarticular persistente, oligoarticular estendida, poliarticular e sistêmica, respectivamente. A probabilidade de remissão clínica sem medicação em 5 anos após o início da remissão foi de 40 e 67 por cento para pacientes com AIJ oligoarticular persistente e sistêmica, respectivamente. Houve associação significante da atividade persistente com o uso combinado de medicações para artrite. A idade de início da AIJ foi o único fator preditivo para remissão clínica (p = 0,002). CONCLUSÃO: Nesta coorte, a probabilidade da AIJ evoluir para remissão clínica foi maior nos subtipos oligoarticular persistente e sistêmico, comparados ao curso poliarticular.

OBJECTIVE: To characterize articular and systemic inflammatory activity in juvenile idiopathic arthritis (JIA), identifying remission status with and without medication. METHODS: A total of 165 JIA cases, followed for a mean period of 3.6 years, were reviewed in order to characterize episodes of inactivity and clinical remission on and off medication. The resulting data were analyzed by means of descriptive statistics, survival analysis, by comparison of Kaplan-Meier curves, log rank testing and binary logistic regression analysis in order to identify predictive factors for remission or persistent activity. RESULTS: One hundred and eight of the cases reviewed fulfilled the inclusion criteria: 57 patients (52.7 percent) exhibited a total of 71 episodes of inactivity, with a mean of 2.9 years per episode; 36 inactivity episodes (50.7 percent) resulted in clinical remission off medication, 35 percent of which were of the persistent oligoarticular subtype. The probability of clinical remission on medication over 2 years was 81, 82, 97 and 83 percent for cases of persistent oligoarticular, extended oligoarticular, polyarticular and systemic JIA, respectively. The probability of clinical remission off medication 5 years after onset of remission was 40 and 67 percent for patients with persistent oligoarticular and systemic JIA, respectively. Persistent disease activity was significantly associated with the use of an anti-rheumatic drug combination. Age at JIA onset was the only factor that predicted clinical remission (p = 0.002). CONCLUSIONS: In this cohort, the probability of JIA progressing to clinical remission was greater for the persistent oligoarticular and systemic subtypes, when compared with polyarticular cases.

Remission status follow-up in children with juvenile idiopathic arthritis.

OBJECTIVE: To characterize articular and systemic inflammatory activity in juvenile idiopathic arthritis (JIA), identifying remission status with and without medication. METHODS: A total of 165 JIA cases, followed for a mean period of 3.6 years, were reviewed in order to characterize episodes of inactivity and clinical remission on and off medication. The resulting data were analyzed by means of descriptive statistics, survival analysis, by comparison of Kaplan-Meier curves, log rank testing and binary logistic regression analysis in order to identify predictive factors for remission or persistent activity. RESULTS: One hundred and eight of the cases reviewed fulfilled the inclusion criteria: 57 patients (52.7%) exhibited a total of 71 episodes of inactivity, with a mean of 2.9 years per episode; 36 inactivity episodes (50.7%) resulted in clinical remission off medication, 35% of which were of the persistent oligoarticular subtype. The probability of clinical remission on medication over 2 years was 81, 82, 97 and 83% for cases of persistent oligoarticular, extended oligoarticular, polyarticular and systemic JIA, respectively. The probability of clinical remission off medication 5 years after onset of remission was 40 and 67% for patients with persistent oligoarticular and systemic JIA, respectively. Persistent disease activity was significantly associated with the use of an anti-rheumatic drug combination. Age at JIA onset was the only factor that predicted clinical remission (p = 0.002). CONCLUSIONS: In this cohort, the probability of JIA progressing to clinical remission was greater for the persistent oligoarticular and systemic subtypes, when compared with polyarticular cases.