Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study

Abstract

Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.

The new 2019-EULAR/ACR classification criteria specific domains at diagnosis can predict damage accrual in 670 childhood-onset systemic lupus erythematosus patients

“…This was a retrospective multicenter study that included a database of 1697 cSLE patients included in the Brazilian cSLE Registry, from 27 Brazilian Pediatric Rheumatology centers, as previously reported. 9,10 One thousand and twenty-seven patients were excluded due to incomplete charts, incomplete data about 1997-ACR or 2019-EULAR/ ACR classification criteria or more than 5 years of disease duration. Therefore, a population of 670 cSLE was studied.…”

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

“…The Brazilian registry assessed 1533 cSLE patients based on three groups with different periods between the onset of signs/symptoms and the diagnosis. This study revealed cSLE patients infrequently had a shorter time interval to diagnosis (< 1 month) characterized by multisystemic, severe, and active conditions, whereas the majority of cSLE patients had a long-time interval (≥ 3 months) to diagnosis, with mild disease onset [ 21 ].…”

Diagnostic Delays and Psychosocial Outcomes of Childhood-Onset Systemic Lupus Erythematosus

“…Individuals diagnosed with cSLE are at a higher risk of irreversible damage than their adult counterparts [24,25]. Timely diagnosis, earlier referral to sub-specialists, and aggressive management improve long-term outcomes, prevent organ failure, and increase survival in these patients [26][27][28][29]. A study conducted in the United States indicated that cSLE patients were less likely to present without delay if they lived in a poor density area for pediatric rheumatologists [28].…”

“…A retrospective cohort study in Brazil divided 1,555 cSLE patients recruited from 27 rheumatology services into three groups based on time to diagnosis: group A: short time interval (<one month), group B: intermediate time interval (one to three months), and group C: long time interval (>three months) [27].…”