Clinical phenotype and outcome in lupus according to age: a comparison between juvenile and adult onset

Fonseca, R.; Aguiar, Francisca; Rodrigues, Mariana; Brito, Iva

doi:10.1016/j.reuma.2016.10.011

Cited by 20 publications

(23 citation statements)

References 13 publications

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“…2,21,24 Also, increased musculoskeletal involvement in adult-onset SLE vs. JSLE patients has been previously demonstrated. 3 , 8 , 13 Tavangar-Rad et al. studied 120 Iranian children with JSLE, and compared age groups in a similar way to the current study (<7, 7–14, and >14 years) and reported more joint involvement with increasing age.…”

Section: Discussionsupporting

confidence: 68%

“…While clinical and laboratory differences between JSLE and adult-onset SLE have been acknowledged, 8 only few and short reports discuss differences within the paediatric age group. 12 , 13 The 418 JSLE patients included in this study allow for more reliable assessment of clinical and laboratory features between the paediatric age groups.…”

Section: Discussionmentioning

confidence: 99%

“…Notably, previous studies did not consider pre-pubertal (≤7) JSLE patients as a distinct age group. 3 , 6 – 8 …”

Section: Discussionmentioning

confidence: 99%

“…Even within the JSLE population, very early disease onset (before the 5th birthday) may be associated with atypical presentations (including fewer autoantibodies), more severe disease course and poor prognosis. 1 , 6 – 8 However, assumptions on variable disease presentation and progression within different JSLE age subgroups are generally based on case reports, case series or relatively small cohorts, 7 , 8 and currently lack scientific evidence from longitudinal national or international studies.…”

Section: Introductionmentioning

confidence: 99%

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Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups

Massias

Smith

Al‐Abadi

et al. 2020

Lupus

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Background Systemic 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. 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 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. Results A 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. Conclusions 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 with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

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Section: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 99%

“…Notably, previous studies did not consider pre-pubertal (≤7) JSLE patients as a distinct age group. 3 , 6 – 8 …”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups

Massias

Smith

Al‐Abadi

et al. 2020

Lupus

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“…7 Although less common, disease severity is higher in jSLE with an increased risk of internal organ involvement especially renal, neuropsychiatric, and hemolytic anemia. [8][9][10][11] Cutaneous manifestations are extremely common in SLE. Skin involvement is found in 72-85% of patients and presented as the first sign in one-quarter.…”

Section: Introductionmentioning

confidence: 99%

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement

Chottawornsak

Rodsaward

Suwannachote

et al. 2018

Lupus

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Objective We aim to explore the differences of skin signs between juvenile- and adult-onset systemic lupus erythematosus and to identify their associations to the development of systemic involvement. Methods A retrospective chart review of 377 systemic lupus erythematosus patients was performed. Results In total, 171 patients with juvenile systemic lupus erythematosus and 206 with adult systemic lupus erythematosus were studied. All patients were of Southeast Asian descent. The mean duration of follow up was 8.18 ± 6.19 and 9.36 ± 7.68 years for juvenile systemic lupus erythematosus and adult systemic lupus erythematosus, respectively. At diagnosis, most patients presented with acute cutaneous lupus erythematosus, whereas chronic cutaneous lupus erythematosus was twice as common in adult systemic lupus erythematosus ( p < 0.001). The mean Systemic Lupus Erythematosus Disease Activity Index of juvenile systemic lupus erythematosus was significantly higher than that of adult systemic lupus erythematosus (14.29 ± 7.13 vs 11.27 ± 6.53). Multivariate analysis revealed the following associations in juvenile systemic lupus erythematosus: acute cutaneous lupus erythematosus and non-scarring alopecia with increased risk of arthralgia, mucosal ulcers with leukopenia, cutaneous vasculitis with seizure, and finding of granular casts. On the contrary, the associations for adult systemic lupus erythematosus were oral ulcers with arthralgia and cutaneous vasculitis with myositis. Conclusions Cutaneous signs in systemic lupus erythematosus may signal prognostic implication. Interestingly, despite similar cutaneous lesions in systemic lupus erythematosus, different ages of onset are associated with different systemic involvement.

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Systemic Lupus Erythematosus in Children and Young People

2021

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Purpose of Review Juvenile-onset systemic lupus erythematosus ((j)SLE) is an autoimmune/inflammatory disease that results in significant damage and disability. When compared to patients with disease onset in adulthood, jSLE patients exhibit increased disease activity, damage and require more aggressive treatments. This manuscript summarises age-specific pathogenic mechanisms and underscores the need for age group–specific research, classification and treatment. Recent Findings Genetic factors play a significant role in the pathophysiology of jSLE, as > 7% of patients develop disease as a result of single gene mutations. Remaining patients carry genetic variants that are necessary for disease development, but require additional factors. Increased ‘genetic impact’ likely contributes to earlier disease onset and more severe phenotypes. Epigenetic events have only recently started to be addressed in jSLE, and add to the list of pathogenic mechanisms that may serve as biomarkers and/or treatment targets. To allow meaningful and patient-oriented paediatric research, age-specific classification criteria and treatment targets require to be defined as currently available tools established for adult-onset SLE have limitations in the paediatric cohort. Summary Significant progress has been made in understanding the pathophysiology of jSLE. Meaningful laboratory and clinical research can only be performed using age group–specific tools, classification criteria and treatment targets.

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Clinical phenotype and outcome in lupus according to age: a comparison between juvenile and adult onset

Cited by 20 publications

References 13 publications

Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups

Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement

Systemic Lupus Erythematosus in Children and Young People

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