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Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

ABSTRACT: Juvenile-onset systemic lupus erythematosus (jSLE) is a multisystemic disease diagnosed in young patients based on the clinical criteria of the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). The importance of this condition lies in its greater aggressiveness compared with lupus diagnosed during adulthood (aSLE). Management, which is based on supportive care and immunosuppressive drugs, aims to reduce the overall disease activity and to prevent exacerbation. Sometimes the onset is accompanied by life-threatening clinical conditions. In this paper, we introduce three recent cases of jSLE that required admission to the Pediatric Intensive Care Unit (PICU) of a Spanish pediatric hospital. This manuscript aims to review some of the main complications associated with jSLE, such as diffuse alveolar hemorrhage, cerebral vasculitis, or an antiphospholipid syndrome; these are life-threatening conditions but they have a chance of favorable prognosis if treated early and aggressively.

SUBMITTER: Huerta-Calpe S

PROVIDER: S-EPMC10217445 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature

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Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

Huerta-Calpe Sergi S Del Castillo-Velilla Ignacio I Felipe-Villalobos Aida A Jordan Iolanda I Hernández-Platero Lluïsa L

Children (Basel, Switzerland) 20230510 5

Juvenile-onset systemic lupus erythematosus (jSLE) is a multisystemic disease diagnosed in young patients based on the clinical criteria of the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). The importance of this condition lies in its greater aggressiveness compared with lupus diagnosed during adulthood (aSLE). Management, which is based on supportive care and immunosuppressive drugs, aims to reduce the overall disease activity and to prevent exacerba ...[more]

PMID: 37238400

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Project description:BackgroundSystemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15-20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study.MethodsPatient 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 American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up.ResultsA total of 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 with 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 with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up.ConclusionsDisease 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 with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

Dataset Information

Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

Publications

Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

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