Background/Purpose: Previous retrospective evaluations suggested that age at diagnosis may influence disease expression in terms of initial clinical presentation, pattern of organ involvement and serological findings in childhood systemic lupus erythematosus (cSLE) patients. However, the small sample size of previous studies, particularly regarding the early-onset cSLE patients (<6 years), precludes a definitive conclusion about their findings. In addition, no data is available regarding the relevance of age in Latin-American c-SLE population. Therefore, the main objective of the present large multicenter study was to compare demographic data, clinical and laboratory findings at disease diagnosis in three different age-related cSLE groups. 

Methods: A retrospective multicenter study was performed in 847 cSLE patients from 10 Pediatric Rheumatology services of São Paulo state, Brazil. Patients were divided in three age-related cSLE groups: 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, to harmonize clinical parameters definition and disease activity tool scoring. Demographic data, clinical and laboratorial features, and disease activity (SLEDAI-2K) were evaluated.

Results:

Groups of cSLE patients were divided: A 39 (4%), B 395 (47%) and C 413 (49%). Of 39 cSLE patients of group A, 3 (8%) were infants, 4 (10%) toddlers and 32 (82%) preschool. Complete C1q deficiency was observed in 3/74 (4%) cSLE patients, all of them of group A. Groups were similar regarding frequencies of nephritis (47% vs. 51% vs. 49%, p=0.742), neuropsychiatric involvement (37% vs. 25% vs. 24%, p=0.348), autoimmune hemolytic anemia (19% vs. 23% vs. 19%, p=0.237), thrombocytopenia (11% vs. 18% vs. 14%, p=0.264) and median of SLEDAI-2K at disease diagnosis [15(5-41) vs. 15(0-58) vs. 14(0-49) p=0.831). Frequencies of female gender, autoantibodies profile, acute phase proteins and low complement levels were also comparable in these three groups (p>0.05). Of note, frequencies of fever (78% vs. 61% vs. 47%, p<0.0001), hepatomegaly (42% vs. 29% vs. 14%, p<0.0001), splenomegaly (28% vs. 12% vs. 4%, p<0.0001) and discoid lupus (13% vs. 4% vs. 4%, p<0.020) were significantly higher in the group A compared to groups B and C. In contrast, the frequencies of weight loss >2kg (19% vs. 28% vs. 36%, p<0.017), photosensitivity (34% vs. 41% vs. 51%, p<0.006), leukopenia<4,000/mm³ (14% vs. 25% vs. 30%, p<0.048) and lymphopenia<1,500/mm³ (22% vs. 41% vs. 47%, p=0.011) were significantly lower in the group A. 

Conclusion:

Our large multicenter study identified that the initial presentation of cSLE is characterized by comparable high frequencies of internal organ involvements and some distinct clinical and laboratorial features in early-onset and adolescent groups.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/features-of-847-childhood-onset-systemic-lupus-erythematousus-patients-in-three-age-related-groups-at-diagnosis-a-brazilian-multicenter-study/