Outcome of 847 Childhood-Onset Systemic LUPUS Erythematousus Patients in Three Age Groups

Sandra R M Lopes¹, Natali W. Gormezano², Roberta C. Gomes Sr.³, Nadia E Aikawa⁴, Rosa M R Pereira⁵, Maria Teresa Terreri⁶, Claudia S. Magalhães⁷, Eunice M. Okuda⁸, Ana Paula Sakamoto⁶, Adriana M E Sallum², Simone Appenzeller⁹, Virgínia Ferriani¹⁰, Cássia M. Barbosa¹¹, Simone Lotufo¹², Adriana A. Jesus^13,14, Luis E C Andrade¹⁵, Lucia M A Campos⁴, Eloisa Bonfa¹⁶ and Clovis A Silva³, ¹Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ²Pediatric Rheumatology Unit, University of São Paulo, São Paulo, Brazil, ³Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ⁴Pediatric Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ⁵Rheumatology Division, Rheumatology Division, Faculdade de Medicina da USP, São Paulo, Brazil, ⁶Pediatric Rheumatology Unit, Federal University of São Paulo, São Paulo, Brazil, ⁷Pediatric Rheumatology Division, São Paulo State University (UNESP), Botucatu, Brazil, ⁸Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil, ⁹Pediatric Rheumatology Unit, State University of Campinas, Campinas, Brazil, ¹⁰Department of Pediatrics School of Medicine of Ribeirão Preto, University of São Paulo (USP-RP), Ribeirão Preto, Brazil, ¹¹Pediatric Rheumatology Unit, Hospital Infantil Darcy Vargas, São Paulo, Brazil, ¹²Pediatric Rheumatology Unit, Hospital Municipal Infantil Menino Jesus, São Paulo, Brazil, ¹³Pediatrics Department, Universidade de São Paulo, São Paulo, Brazil, ¹⁴Pediatrics, Instituto da Criança da Faculdade de Medicina da Universidade de São Paulo (FMUSP), Sao Paulo, Brazil, ¹⁵Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ¹⁶Rheumatology Divison, Hospital das Clinicas, Faculdade de Medicina, University of São Paulo, São Paulo, Brazil

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Systemic lupus erythematosus (SLE)

Session Information

Date: Monday, November 14, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster II: Myositis, Systemic Lupus Erythematosus, Sjögren's Syndrome

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.

Disclosure: S. R. M. Lopes, None; N. W. Gormezano, None; R. C. Gomes Sr., None; N. E. Aikawa, None; R. M. R. Pereira, None; M. T. Terreri, None; C. S. Magalhães, None; E. M. Okuda, None; A. P. Sakamoto, None; A. M. E. Sallum, None; S. Appenzeller, None; V. Ferriani, None; C. M. Barbosa, None; S. Lotufo, None; A. A. Jesus, None; L. E. C. Andrade, None; L. M. A. Campos, None; E. Bonfa, None; C. A. Silva, CNPq, 2.

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 .

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