scholarly journals Unique Characteristics of Prepubertal Onset Systemic Lupus Erythematosus

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
R. Abdwani ◽  
E. Abdalla ◽  
I. Al-Zakwani

Objectives. The aim of this study was to investigate the influence of age at disease onset on disease expression and outcomes of pediatric systemic lupus erythematosus SLE (pSLE). Methods. A total of 103 patients with pSLE from Sultan Qaboos University Hospital, Oman, were retrospectively studied. Epidemiological, clinical phenotype, disease severity, serology, treatment, and outcome were compared among the three groups using univariate statistical tests. Results. The mean disease duration of the cohort was 9.8 ± 4.7 years. The patients were divided into three groups: prepubertal onset (n=39) with mean age at diagnosis of 5.1 ± 2.0 years and pubertal disease onset (n=29) with mean age at diagnosis of 10.8 ± 1.0 years as well as postpubertal disease onset (n=35) group with mean age at diagnosis of 15.3 ± 1.6 years. The prepubertal pSLE cohort demonstrates unique characteristics with increased frequency of familial SLE (61%) of which 49% were from first-degree relatives. Similarly, this group had distinctive clinical features, which included increased renal disease in pubertal and postpubertal groups, respectively (51% vs 23% vs 20%; p=0.039). Prepubertal, similar to pubertal group, had a higher incidence of cutaneous manifestations than in the postpubertal group (74% vs 69% vs 46%; p=0.029). Laboratory features in prepubertal group were distinct with increased frequency of positive anti-cardiolipin antibodies (47%), anti-glycoprotein antibodies (42%), ANCA (62%), and low complement levels (97%) compared to pubertal and postpubertal group. The prepubertal group also has the lowest frequency of positive SSA antibodies (18%) and SSB antibodies (5.1%). The overall mean SLEDAI score in pSLE cohort was 15.6 ± 18.5. The mean SLEDAI scores among the groups showed no significance difference (p=0.110). The overall SLICC DI ≥1 was 36% with a mean damage score of 0.76 ± 1.38. No significant differences in damage index (SLICC DI ≥1) were noted among the groups. Conclusions. Distinct clinical features were identified in prepubertal onset pSLE population of Arab ethnicity. Given the high rate of consanguineous marriage and high frequency of familial SLE in this cohort, these manifestations could be explained by higher frequency of genetic factors that influence the disease pathogenesis.

Lupus ◽  
2020 ◽  
Vol 29 (13) ◽  
pp. 1815-1820
Author(s):  
Luciana Gonzalez Lucero ◽  
Ana L Barbaglia ◽  
Verónica I Bellomio ◽  
Maria C Bertolaccini ◽  
Maximiliano A Machado Escobar ◽  
...  

The aim of this study was to determine the prevalence and incidence of Systemic Lupus Erythematosus (SLE) in Tucumán, Argentina. Methods: The study included inpatient and outpatient charts from four public hospitals and private practice rheumatology clinics, all of them members of the Tucumán Rheumatology Society. Patients older than 16 years with diagnosis of SLE between January 2005 and December 2012 were included. Prevalence and annual incidence were calculated as the number of cases per 100.000 inhabitants during the period 2005 to 2012. Results: Three hundred fifty-three patients were identified. The mean age at diagnosis was 30.5 ± 11.7 years, 93.5% women, 83% mestizos. Prevalence was 24.3 cases/100.000 inhabitants (CI 95% 22.6–28.8) and age-adjusted (≥16 years) of 34.9 cases/100.000 inhabitants (CI 95% 32.8–41.1). The annual incidence in 2005 was 1.8 cases/100.000 inhabitants (95% CI 1–2.9) and 2012 of 4.2 cases/100.000 inhabitants (95% CI 2.9–5.8). Mortality was 9.1%, with infections being the most frequent cause (14/32). Conclusion: The prevalence of SLE in the province of Tucumán was 34.9 cases/100.000 inhabitants.


Lupus ◽  
2021 ◽  
pp. 096120332110524
Author(s):  
Cristina Reategui-Sokolova ◽  
Rocío Violeta Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos-Miranda ◽  
Paola Alejandra Zeña-Huancas ◽  
...  

Objectives: This study aims to determine the factors associated with absenteeism, presenteeism, and overall work impairment in patients with systemic lupus erythematosus (SLE). Methods: A total of 133 consecutive working patients with SLE were assessed between October 2017 and December 2018, using a standardized data collection form. Sociodemographic, disease, and work-related variables were collected. Work productivity and activity impairment (WPAI) was assessed with the respective questionnaire; absenteeism and presenteeism due to overall health and symptoms during the past 7 days were scored. Linear regression models were performed to determine the factors associated with absenteeism, presenteeism, and overall work impairment. Potential factors included were age at diagnosis, gender, socioeconomic status, educational level, SLEDAI, SLICC/ACR damage index (SDI), FACIT-Fatigue, and the domains of the LupusQoL Results: The mean age at diagnosis was 32.2 years (11.8); 121 (91.7%) were female. Nearly all patients were Mestizo. The mean percent of time for absenteeism was 5.0 (12.9), it was 28.5 (26.4) for presenteeism, and it was 31.3 (27.2) for overall work impairment. In the multiple regression analysis, factors associated with absenteeism were disease duration (B = −0.34; SE = 0.12; p = 0.007), pain (B = −0.14; SE = 0.06; p = 0.046), intimate relationship (B = −0.07; SE = 0.03; p = 0.046), and emotional health (B = 0.16; SE = 0.06; p = 0.006); factors associated with presenteeism were physical health (B = −0.43; SE = 0.14; p = 0.002) and FACIT (B = −0.87; SE = 0.30; p = 0.005); and factors associated with overall work impairment were pain (B = −0.40; SE = 0.11; p = 0.001) and FACIT-Fatigue (B = −0.74; SE = 0.28; p = 0.010). Conclusion: A poor HRQoL and higher levels of fatigue were associated with a higher percentage of absenteeism, presenteeism, and overall work impairment in SLE patients.


Lupus ◽  
2020 ◽  
Vol 29 (12) ◽  
pp. 1644-1649
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reátegui-Sokolova ◽  
Rocío V Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  

Objective To define the factors associated with fatigue in Mestizo patients with Systemic Lupus Erythematosus (SLE). Methods This is a cross-sectional study of SLE patients from a single center cohort. Visits were performed every six months. For these analyses, the first visit between October 2017 and December 2018 was included. Demographic and clinical characteristics as well as treatment were recorded at every visit. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-FT), Health-Related Quality of Life (HRQoL) with the LupusQoL, disease activity with the Systemic Lupus Erythematosus Disease Activity Index –2 K (SLEDAI-2K), and damage with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). Prednisone use was recorded as current daily dose. Immunosuppressive drugs and antimalarial use were recorded as current, past or never. Univariable and multivariable analyses were performed using linear regression models. For the multivariable analyses, model selection followed a backward elimination procedure. Results Two hundred and twenty-six patients were evaluated. The mean (SD) age at diagnosis was 35.6 (13.1) years, 211 (93.4%) were female; and disease duration was 11.0 (7.3) years. The mean SLEDAI and SDI were 2.4 (3.5) and 1.3 (1.5), respectively. The mean FACIT-FT was 33.1 (10.8). On the multivariable analysis, age at diagnosis and some domains of HRQoL (physical health, emotional health and fatigue) remained associated. Conclusions Age at diagnosis is negatively associated with fatigue whereas HRQoL domains like physical health, emotional health and fatigue are positively associated with fatigue.


2021 ◽  
Vol 15 (1) ◽  
pp. 35-39
Author(s):  
Sadaf Andleeb ◽  
Tafazzul-E-Haque Mahmud ◽  
Aflak Rasheed ◽  
Muhammad Shahid Mehmood ◽  
Iram Gull ◽  
...  

Background: Early diagnosis and effective treatment in systemic lupus erythematosus (SLE) has very crucial role. Anti dsDNA is very important diagnostic tool and activity marker in SLE. This study aimed to determine the association of anti dsDNA antibodies titer with non-renal manifestations of systemic lupus erythematosus and systemic lupus erythematosus disease activity index (SLEDAI). Patients and methods: It was a cross-sectional study and was carried out at Department of Rheumatology and Immunology, Tertiary Care Hospital, Lahore from Feb 2021 to May 2021. The study involved 69 male and female patients satisfying the systemic lupus international collaborating clinics (SLICC) classification criteria. Questions regarding different symptoms were asked and disease activity parameters were noted excluding renal parameters. Anti-dsDNA titers were measured from standard laboratory using immunofluorescence technique and were correlated with SLEDAI score. A written informed consent was obtained from every patient. Results: The mean age of the patients was 30.7±10.2 years while the mean duration of disease 1.94±2.65 years. We observed a female predominance among these patients with male to female ratio of 1:7.6. There were fifty-four (78.3%) patients with active disease. The mean anti-dsDNA levels were significantly higher in patients with active disease (315.73±481.68 vs. 78.46±113.64 IU/mL; p-value=0.003). There was a significantly strong positive correlation between anti-dsDNA levels and SLEDAI score (r=0.358; p-value=0.006). When compared, significant difference was observed in mean anti-dsDNA titers in patients with chronic cutaneous manifestations (p-value=0.040), lymphopenia (p-value= 0.012), pleurisy/pericarditis (p-value= 0.024) and leukopenia <3000/mm3 (p-value= 0.001). Conclusion: Anti-dsDNA antibodies titers are remarkably increased in patients with non-renal manifestations of systemic lupus erythematosus particularly with chronic cutaneous manifestations and leukopenia and positively correlate with disease activity status and SLEDAI score.


Lupus ◽  
2021 ◽  
pp. 096120332110345
Author(s):  
Stefan Vordenbäumen ◽  
Alexander Sokolowski ◽  
Anna Rosenbaum ◽  
Claudia Gebhard ◽  
Johanna Raithel ◽  
...  

Objective Hypomethylation of CD40-ligand (CD40L) in T-cells is associated with increased disease activity in systemic lupus erythematosus (SLE). We therefore investigated possible associations of dietary methyl donors and products with CD40L methylation status in SLE. Methods Food frequency questionnaires were employed to calculate methyl donor micronutrients in 61 female SLE patients (age 45.7 ± 12.0 years, disease duration 16.2 ± 8.4 years) and compared to methylation levels of previously identified key DNA methylation sites (CpG17 and CpG22) within CD40L promotor of T-cells using quantitative DNA methylation analysis on the EpiTYPER mass spectrometry platform. Disease activity was assessed by SLE Disease Activity Index (SLEDAI). Linear regression modelling was used. P values were adjusted according to Benjamini & Hochberg. Results Amongst the micronutrients assessed (g per day), methionine and cysteine were associated with methylation of CpG17 (β = 5.0 (95%CI: 0.6-9.4), p = 0.04; and β = 2.4 (0.6-4.1), p = 0.02, respectively). Methionine, choline, and cysteine were additionally associated with the mean methylation of the entire CD40L (β = 9.5 (1.0-18.0), p = 0.04; β = 1.6 (0.4-3.0), p = 0.04; and β = 4.3 (0.9-7.7), p = 0.02, respectively). Associations of the SLEDAI with hypomethylation were confirmed for CpG17 (β=-32.6 (-60.6 to -4.6), p = 0.04) and CpG22 (β=-38.3 (-61.2 to -15.4), p = 0.004), but not the mean methylation of CD40L. Dietary products with the highest impact on methylation included meat, ice cream, white bread, and cooked potatoes. Conclusions Dietary methyl donors may influence DNA methylation levels and thereby disease activity in SLE.


Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.


Lupus ◽  
2021 ◽  
pp. 096120332110050
Author(s):  
Rory C Monahan ◽  
Liesbeth JJ Beaart-van de Voorde ◽  
Jeroen Eikenboom ◽  
Rolf Fronczek ◽  
Margreet Kloppenburg ◽  
...  

Introduction We aimed to investigate risk factors for fatigue in patients with systemic lupus erythematosus (SLE) and neuropsychiatric symptoms in order to identify potential interventional strategies. Methods Patients visiting the neuropsychiatric SLE (NPSLE) clinic of the Leiden University Medical Center between 2007–2019 were included. In a multidisciplinary consensus meeting, SLE patients were classified as having neuropsychiatric symptoms of inflammatory origin (inflammatory phenotype) or other origin (non-inflammatory phenotype). Fatigue was assessed with the SF-36 vitality domain (VT) since 2007 and the multidimensional fatigue inventory (MFI) and visual analogue scale (VAS) since 2011. Patients with a score on the SF-36 VT ≥1 standard deviation (SD) away from the mean of age-related controls of the general population were classified as fatigued; patients ≥2 SD away were classified as extremely fatigued. Disease activity was measured using the SLE disease activity index-2000. The influence of the presence of an inflammatory phenotype, disease activity and symptoms of depression and anxiety as measured by the hospital anxiety and depression scale (HADS) was analyzed using multiple regression analyses corrected for age, sex and education. Results 348 out of 371 eligible patients filled in questionnaires and were included in this study . The majority was female (87%) and the mean age was 43 ± 14 years. 72 patients (21%) had neuropsychiatric symptoms of an inflammatory origin. Fatigue was present in 78% of all patients and extreme fatigue was present in 50% of patients with an inflammatory phenotype vs 46% in the non-inflammatory phenotype. Fatigue was similar in patients with an inflammatory phenotype compared to patients with a non-inflammatory phenotype on the SF-36 VT (β: 0.8 (95% CI −4.8; 6.1) and there was less fatigue in patients with an inflammatory phenotype on the MFI and VAS (β: −3.7 (95% CI: −6.9; −0.5) and β: −1.0 (95% CI −1.6; −0.3)). There was no association between disease activity and fatigue, but symptoms of anxiety and depression (HADS) associated strongly with all fatigue measurements. Conclusion This study suggests that intervention strategies to target fatigue in (NP)SLE patients may need to focus on symptoms of anxiety and depression rather than immunosuppressive treatment.


Sign in / Sign up

Export Citation Format

Share Document