Background/Purpose: Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Up to 20% of patients develop symptoms before their 18^th birthday and are diagnosed with juvenile-onset SLE (JSLE)(1,2). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE(1,3-5). This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study.

Methods: Patient records were accessed and grouped based on age at disease-onset: pre-pubertal (≤7 years), peri-pubertal (8-13 years) and adolescent (14-18 years). The presence of ACR criteria, laboratory results, disease activity (pBILAG2004) and damage (SLICC damage index) were evaluated at diagnosis and last follow-up.

Results: 418 JSLE patients were included in this study: 43(10.3%) with pre-pubertal disease onset; 240(57.4%) with peri-pubertal onset, and 135(32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared to pre-pubertal and peri-pubertal patients (pBILAG2004 scores: 9[4-20] vs. 7[3-13] vs. 7[3-14] respectively, p=0.015) with increased activity in the following BILAG domains: mucocutaneous (p=0.025), musculoskeletal (p=0.029), renal (p=0.027), and cardiorespiratory (p=0.001). Furthermore, adolescent JSLE patients were more frequently ANA positive (p=0.034) and exhibited higher anti-dsDNA titres (p=0.001). Pre-pubertal individuals less frequently presented with leukopenia (p=0.002), thrombocytopenia (p=0.004) or low complement (p=0.002) when compared to other age groups. No differences were identified in disease activity (pBILAG2004), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow-up.

Conclusion: Disease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and “classic” autoantibody, immune cell and complement patterns when compared to younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups. 

References:

1. Hedrich CM, et al. Juvenile-onset systemic lupus erythematosus (jSLE) – Pathophysiological concepts and treatment options. Best Pract Res Clin Rheumatol. 2017;31(4):488-504.
2. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-21.
3. Chen YM, et al. Onset age affects mortality and renal outcome of female systemic lupus erythematosus patients: a nationwide population-based study in Taiwan. Rheumatology (Oxford). 2014;53(1):180-5.
4. Descloux E, et al. Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus. Rheumatology (Oxford). 2009;48(7):779-84.
5. Fonseca R, et al. Clinical phenotype and outcome in lupus according to age: a comparison between juvenile and adult onset. Reumatol Clin. 2018;14(3):160-3.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-laboratory-characteristics-in-juvenile-onset-systemic-lupus-erythematosus-across-age-groups/