Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: A recent large study comparing childhood systemic lupus erythematosus (cSLE) with adult-onset SLE revealed a more aggressive and worse outcome in the former group. However, analysis of age-related differences regarding outcome focused on pediatric patients and particularly assessing early-onset cSLE patients (<6 years) is limited to very small sample size precluding a definitive conclusion about their findings. Therefore, the objective of this large multicenter study was to compare cumulative clinical/laboratory features, disease damage and death in three different age groups.
Methods: A retrospective multicenter study was performed in 847 cSLE patients from 10 Pediatric Rheumatology Divisions of São Paulo State, Brazil. Patients were divided in three age-related cSLE groups at diagnosis: A – early-onset (<6 years), B – school age (≥6 and <12 years) and C – adolescent (≥12 and < 18 years). An investigator meeting was held to define the protocol and to standardize disease parameters definitions. Demographic data, clinical and laboratorial features, disease damage (SLICC/ACR-DI) and death were evaluated at last visit.
Results: Groups of cSLE patients were divided according to the age at diagnosis: A=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 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 (5% vs. 3% vs. 0.3%,p=0.008) were more often observed in group A compared to B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, hemolytic anemia and vasculitis were alike in all groups(p>0.05). Death was significantly higher in group A compared to groups B and C (15% vs. 10% vs. 6%,p=0.028). Among 69 deaths, 8/33(24%) occurred at first month after diagnosis. Infection accounted for 54/69 (78%) of the overall deaths and 38/54 (70%) had concomitant disease activity. Other causes of death were nephritis (acute kidney injury or chronic renal disease) in 6 (9%), alveolar hemorrhage in 3 (4%), massive intracranial bleeding in 1 (1.4%), multiple thrombosis due to catastrophic antiphospholipid syndrome in 1 (1.4%), B-cell lymphoma in 1 (1.4%) and unknown 3 (4%).
Conclusion: 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 damages in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE was an early event mainly attributed to infection with associated disease activity.
To cite this abstract in AMA style:Lopes SRM, Gormezano NW, Gomes RC Sr., Aikawa NE, Pereira RMR, Terreri MT, Magalhães CS, Okuda EM, Sakamoto AP, Sallum AME, Appenzeller S, Ferriani V, Barbosa CM, Lotufo S, Jesus AA, Andrade LEC, Campos LMA, Bonfa E, Silva CA. Outcome of 847 Childhood-Onset Systemic LUPUS Erythematousus Patients in Three Age Groups [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/outcome-of-847-childhood-onset-systemic-lupus-erythematousus-patients-in-three-age-groups/. Accessed July 23, 2019.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcome-of-847-childhood-onset-systemic-lupus-erythematousus-patients-in-three-age-groups/