scholarly journals Disease characteristics in patients with juvenile- and adult-onset systemic lupus erythematosus: A multi-center comparative study

2021 ◽  
Author(s):  
Sherif M Gamal ◽  
Nermeen Fouad ◽  
Nora Yosry ◽  
Wael Badr ◽  
Nesreen Sobhy
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mortality Rate ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Disease Characteristics ◽  
Onset Systemic Lupus Erythematosus

Objectives: This study aims to compare disease characteristics in patients with juvenile-onset systemic lupus erythematosus (JSLE) and adult-onset systemic lupus erythematosus (ASLE). Patients and methods: Between June 2010 and March 2020, a total of 186 patients with JSLE (23 males, 163 females; median age: 25 years; range, 20 to 30.3 years) and 236 patients with ASLE (23 males, 213 females; median age: 35 years; range, 29 to 40 years) were retrospectively analyzed. Clinical and laboratory data, treatment received, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index (SDI) scores, comorbidities and deaths were compared between the groups. Results: The JSLE patients showed statistically significant higher constitutional manifestations, cardiac manifestations, serositis, nephritis, end-stage renal disease, neurological manifestations, gastrointestinal manifestations, secondary vasculitis, Raynaud’s, livedo-reticularis, dry mouth, dry eye, ocular manifestations, avascular necrosis, hematological manifestations, and hypocomplementemia (p<0.001, p=0.016, p=0.005, p=0.001, p=0.04, p<0.001, p<0.001, p<0.001, p=0.002, p=0.043, p=0.004, p=0.03, p<0.001, p=0.01, p<0.001, and p=0.001, respectively). Median SLEDAI scores were statistically significant higher in the JSLE group, both at onset (p<0.001) and in the final follow-up visit (p<0.001). Median SLICC scores were also higher in the JSLE group (p<0.001). Mycophenolate mofetil and intravenous pulse steroids were more frequently used in the juvenile group (p<0.001 and p=0.03, respectively). Hypertension, dyslipidemia, and avascular necrosis were found to be statistically significantly higher in the JSLE group (p<0.001, p=0.006, and p=0.01, respectively). The mortality rate was statistically significantly higher in the JSLE group than the ASLE group (p<0.001). Conclusion: The JSLE patients showed more serious manifestations, higher disease activity, higher damage index, and mortality rate compared to ASLE patients. These results suggest the need of a regular follow-up and close surveillance of JSLE patients.

Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study

2016 ◽  
Vol 43 (8) ◽  
pp. 1490-1497 ◽  
Author(s):  
Young Bin Joo ◽  
So-Yeon Park ◽  
Soyoung Won ◽  
Sang-Cheol Bae
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Hemolytic Anemia ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Adult Onset ◽  
Single Center ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Objective.To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort.Methods.A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models.Results.After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6–35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1–3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE.Conclusion.The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.

scholarly journals POS0725 CLINICAL AND IMMUNOLOGICAL CHARACTERISTICS OF PATIENTS WITH JUVENILE-, ADULT- AND LATE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 612.2-613
Author(s):  
O. Iaremenko ◽  
D. Koliadenko ◽  
I. Matiyashchuk
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Disease Onset ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Autoantibody Profile ◽  
Onset Systemic Lupus Erythematosus

Background:Systemic lupus erythematosus (SLE) predominantly develops in women of child-bearing age. However, nearly 20% of cases present during childhood, generally after puberty (juvenile-onset SLE, JSLE). On the other hand, 10-20% of patients develop SLE after the age of 45-50 years (late-onset SLE, LSLE) [1]. It is known that age at disease onset can influence the clinical presentation and course of SLE, but the findings are not always consistent across the studies [2].Objectives:The aim of this study was to evaluate the spectrum of clinical manifestations and autoantibody profile in patients with SLE in the central region of Ukraine regarding age at onset.Methods:The study included 258 SLE patients before starting an adequate therapy, comprising 225 females (87.2%) and 33 males (12.8%). The median age at SLE onset was 28 (20-39) years. The patients were classified into 3 groups: I – age at SLE onset ≤18 years (JSLE; n=52; 20.2%), II – SLE onset at age 19-44 years (adult-onset SLE, ASLE; n=161; 62.4%), III – age at disease onset ≥45 years (LSLE; n=45; 17.4%). The clinical and demographic data, SLE Disease Activity Index (SLEDAI), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and autoantibody profile were analyzed. Quantitative and categorical data were compared using Kruskal-Wallis test and chi-square test, respectively.Results:There was a difference in prevalence of malar rash between the groups (p=0.022): it was more common in JSLE (40.4%) and ASLE (34.4%) than in LSLE patients (15.6%; p=0.04 and 0.05, respectively). Similar distribution was found for renal involvement: JSLE and ASLE patients presented higher rates of nephritis (55.8% and 49.4%, respectively) than LSLE patients (23.8%; p=0.012 and 0.014, respectively). But the groups did not differ significantly with regard to nephrotic syndrome (p=0.224). ASLE was associated with more frequent alopecia (38.8%) comparing with JSLE (19.2%; p=0.04). Moreover, ASLE patients also had the highest frequency of lymphadenopathy (56.3%) whereas in LSLE it was observed only in 25.0% of patients (p=0.001). Serositis was more common in LSLE (54.5%) and ASLE (43.8%) than in JSLE (23.1%; p=0.011 and 0.034, respectively). Although secondary Sjögren’s syndrome was more frequently observed in ASLE (7.6%) and LSLE (7.3%) than in JSLE (0.0%), the difference did not achieve statistical significance (p=0.157). Also, no differences were observed in the occurence of arthritis, pulmonary and neurological manifestations, constitutional symptoms, SLEDAI score among the groups. Median CRP level in LSLE was significantly higher (14.0 (1.1-46.4) mg/L) than in JSLE (0.7 (0.0-12.0) mg/L) (p<0.05). But all groups did not differ significantly with regard to ESR levels. When differences in antinuclear antibodies were analyzed, we disclosed that the frequency of anti-dsDNA positive results was significantly higher in JSLE (68.6%) and ASLE (70.1%) patients when compared with that found in LSLE patients (31.3%) (p=0.016 and 0.001, respectively). There were no significant differences between groups with regard to positivity for other antibodies (anti-Sm, -Ro, -La, -RNP, antiphospholipid antibodies).Conclusion:JSLE and ASLE patients are more likely to have malar rash, nephritis and anti-dsDNA positivity. Alopecia and lymphadenopathy are most frequent in ASLE patients. JSLE are far less likely to have serositis than any other group. Patients with LSLE demonstrate comparatively low frequency of major organ involvement, but they have higher levels of CRP.References:[1]Ambrose N., et al. Differences in disease phenotype and severity in SLE across age groups. Lupus. 2016;25(14):1542-1550.[2]Livingston B., et al. Differences in autoantibody profiles and disease activity and damage scores between childhood- and adult-onset systemic lupus erythematosus: a meta-analysis. Seminars in Arthritis and Rheumatism. 2012;42(3):271-280.Disclosure of Interests:None declared

Cytokines, 25-OH vit D and disease activity in patients with juvenile-onset systemic lupus erythematosus

Lupus ◽  
2020 ◽  
pp. 096120332097306
Author(s):  
Assem M Abo-Shanab ◽  
Shams Kholoussi ◽  
Rania Kandil ◽  
Dalia Dorgham
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Negative Correlation ◽  
Lupus Erythematosus ◽  
Laboratory Data ◽  
Damage Index ◽  
Systemic Lupus ◽  
Juvenile Onset ◽  
Ifn Γ ◽  
Onset Systemic Lupus Erythematosus

Background Juvenile onset systemic lupus erythematosus JO-SLE patients usually exhibit a more aggressive disease course compared to adult patients. Vitamin D deficiency is proposed to be associated with increased disease activity and flares of numerous autoimmune diseases like SLE, rheumatoid arthritis, and scleroderma. Objective To evaluate the level of IL-17, IFN-γ, and 25-OH Vit D in JO-SLE patients versus healthy controls, and determine the correlation of those inflammatory mediators with SLE disease activity and damage scores. Furthermore, to analyze the relationship between 25-OH Vit D levels with the inflammatory cytokines (IFN-γ and IL-17) in JO-SLE patients. Patients and methods Fifty JO-SLE patients and 25 controls were included in this study. Clinical and laboratory data of patients at the time of the study were recorded. SLE disease activity and damage were assessed using the SLEDAI-2K disease score and SLICC damage index, respectively. Plasma 25-OH Vit D, IFN-γ, and IL-17 concentrations were determined using the human ELISA kit. Results Plasma 25-OH Vit D levels (20 ng/mL) were significantly lower in JO-SLE patients compared to (31 ng/mL) controls (P = 0.014). Plasma levels of IFN-γ and IL-17 were significantly higher (163.5 and 25.5 pg./mL) in JO-SLE patients than (68.3 and 3 pg./mL) that of controls (P = 0.016 and P = 0.013). There was a significant negative correlation between 25-OH Vit D levels and SLEDAI-2K (R= -0.431) as well as IFN-γ (R= -0.471) plasma level (P = 0.022 and P = 0.027). Conclusion IFN-γ and IL-17 were significantly higher in JO-SLE patients, while 25-OH Vit D was significantly lower compared to controls. There was a negative correlation between 25-OH Vit D and each of SLEDAI-2K and IFN-γ.

scholarly journals Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups

Lupus ◽  
2017 ◽  
Vol 26 (9) ◽  
pp. 996-1001 ◽  
Author(s):  
S R M Lopes ◽  
N W S Gormezano ◽  
R C Gomes ◽  
N E Aikawa ◽  
R M R Pereira ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mortality Rate ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Early Onset ◽  
Age Groups ◽  
Damage Index ◽  
Concomitant Disease ◽  
Systemic Lupus ◽  
Group A

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1–23.4) vs 6.2 (0–17) vs 3.3 (0–14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0–9) vs 0 (0–6) vs 0 (0–7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

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

Lupus ◽  
2020 ◽  
Vol 29 (5) ◽  
pp. 474-481 ◽  
Author(s):  
J S Massias ◽  
E M D Smith ◽  
E Al-Abadi ◽  
K Armon ◽  
K Bailey ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Onset ◽  
Age Groups ◽  
Damage Index ◽  
Systemic Lupus ◽  
Juvenile Onset ◽  
Acr Criteria

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.

scholarly journals Fatores Determinantes de Morbilidade nos Doentes com Lúpus Eritematoso Sistémico

2017 ◽  
Vol 30 (5) ◽  
pp. 368
Author(s):  
Margarida Jacinto ◽  
Eliana Silva ◽  
Nuno Riso ◽  
Maria Francisca Moraes-Fontes
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Damage Index ◽  
Disease Activity Index ◽  
Systemic Lupus Erythematosus Disease ◽  
Systemic Lupus ◽  
American College Of Rheumatology

Introduction: Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease’s prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis.Objective: To test damage determinants in a single-centre systemic lupus erythematosus cohort.Material and Methods: Retrospectively followed systemic lupus erythematosus female patients (defined by the identification of at least four systemic lupus erythematosus American College of Rheumatology criteria – fulfillment 100%, n = 76) over the past five years. Age of onset, ethnicity, disease duration, number of American College of Rheumatology criteria at the end of follow-up, cumulative: renal, neuropsychiatric and articular phenotypes, hypertension, dyslipidaemia, smoking and Systemic Lupus Erythematosus Disease Activity Index 2K were correlated to the presence and degree of irreversible damage (Systemic Lupus International Collaborating Clinics Damage Index). Accumulation of American College of Rheumatology criteria was measured in a sub-group of patients followed from disease onset (within a year of the first symptom ascribed to systemic lupus erythematosus) (n = 39 – 51%); Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index were performed. Statistical analysis was performed using Chi-square, Wilcoxon Mann-Whitney tests and Spearman correlation rho (Sig. 2-tailed p < 0.05).Results: Systemic Lupus International Collaborating Clinics/Systemic Damage Index > 0 was present in 56.6% and significantly associated to a longer duration, a higher number of American College of Rheumatology criteria and a neuropsychiatric phenotype when compared with those with no damage. The final number of American College of Rheumatology criteria accrued was positively correlated to a higher disease activity over the past five years of follow-up (Spearman´s rho 0.02 and p < 0.05). There was no effect from other features.Discussion and Conclusion: Disease duration and number of American College of Rheumatology criteria predict Systemic Lupus International Collaborating Clinics/ Systemic Damage Index. neuropsychiatric disease has an impact on damage accrual.

scholarly journals Differences in long-term disease activity and treatment of adult patients with childhood- and adult-onset systemic lupus erythematosus

2008 ◽  
Vol 61 (1) ◽  
pp. 13-20 ◽  
Author(s):  
Aimee O. Hersh ◽  
Emily von Scheven ◽  
Jinoos Yazdany ◽  
Pantelis Panopalis ◽  
Laura Trupin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Adult Patients ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus
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