Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups

Abstract

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America

“…LA includes 33 countries and dependencies, a large population, two main languages (Spanish and Portuguese), with distinct socio-economic and political status. Two groups have reported data focused on epidemiology, clinical and laboratorial features of cSLE patients in LA: BRAC-SLE (Brazilian Childhood-onset SLE Registry Group) [2][3][4][5][6][7][8][9][10][11][12][13][14][15] and GLADEL (Grupo Latino Americano De Estudio del Lupus) [16][17][18][19].…”

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study

“…This study was conducted in 10 Pediatric Rheumatology services in the state of São Paulo, Brazil including a population of 847 cSLE patients [8]. All patients fulfilled the American College of Rheumatology (ACR) criteria for SLE [9], with disease onset before the age of 18.…”

“…Descriptors of SLE Disease Activity Index 2000 (SLE-DAI-2 K) were used to define clinical manifestations [10], and custom definitions as previously reported [8]. Cumulative clinical manifestations included constitutional involvement [defined as fever and lymphadenopathy (peripheral lymph node enlargement > 1.0 cm)], hepatomegaly [based on physical exam with liver edge ≥2 cm below the right costal margin or imaging (ultrasound or computer tomography when available)], splenomegaly [based on physical exam with palpable spleen or imaging (ultrasound or computer tomography when available)], musculoskeletal involvement, serositis, neuropsychiatric and renal involvement.…”

Comparison of Early-Onset and Late-Onset Pediatric Systemic Lupus Erythematosus

“…Aynı Brezilya grubunun başka bir çalışmasında, bu kez 3 grubun belirli bir süre sonundaki mortalite oranları, hem toplam hasar skorları hem de tek tek organ hasar sıklıkları karşılaştırılmıştır. 11 SLICC/ACR hasar indeksine göre, mediyan hasar skorları arasında fark olmamasına rağmen; tek tek bakıldığında nöropsikiyatrik, cilt ve periferik vasküler hasarın erken başlangıçlı grupta (<6 yaş) diğer gruplara göre anlamlı oranda daha fazla görülmüştür. Yine mortalite oranının, bu grupta diğer gruplara göre oldukça fazla olduğu saptanmıştır (<6 yaş: %15, 6-12 yaş: %10, >12 yaş: %6).…”

“…Prepubertal hastalarda hastalığın daha şiddetli seyrettiğini bildiren çalışmalar olduğu gibi, her iki grup arasında hastalık aktivitesi ve organ tutulumları açısından bir fark olmadığını bildiren çalışmalar da mevcuttur. [7][8][9][10][11] Bu çalışmalara kadar, pediatrik SLE insidansının çoğunlukla hormonal etkiyle puberte ve sonrasında zirve yaptığı ve bu nedenle hastaların çoğunlukla adölesan olduğu öne sürülmüştür. Ancak, bu çalışmalarda, prepubertal ve postpubertal başlangıçlı hastaların sayısının çoğunlukla birbirine çok yakın olduğu görülmüştür.…”