From Childhood to Adulthood: Disease Activity Trajectories in Childhood-Onset Systemic Lupus Erythematosus

2018 ◽  
Vol 70 (5) ◽  
pp. 750-757 ◽  
Author(s):  
Lily Siok Hoon Lim ◽  
Eleanor Pullenayegum ◽  
Brian M. Feldman ◽  
Lillian Lim ◽  
Dafna D. Gladman ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Hepatitis A virus vaccination in childhood-onset systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 28 (2) ◽  
pp. 234-240 ◽  
Author(s):  
S Mertoglu ◽  
S Sahin ◽  
O F Beser ◽  
A Adrovic ◽  
K Barut ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Healthy Subjects ◽  
Hepatitis A ◽  
Hepatitis A Virus ◽  
Activity Index ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Objectives: Vaccination of systemic lupus erythematosus patients with non-live vaccines may decrease vaccine-preventable infections and mortalities. In the present study, we aimed to compare the immunogenicity and safety of inactivated hepatitis A vaccination in childhood-onset systemic lupus erythematosus and healthy subjects. Methods: A total of 30 childhood-onset systemic lupus erythematosus and 39 healthy participants who were seronegative for hepatitis A received two doses of the hepatitis A vaccine in a 0- and 6-month schedule. Hepatitis A virus (HAV) IgG antibodies were measured before vaccination and 7 months after the vaccination. Results: Although anti-HAV IgG antibody titers after vaccination were found to be somewhat lower in children with systemic lupus erythematosus than that of the healthy subjects ( p < 0.05), the difference in seroconversion rate was insignificant between childhood-onset systemic lupus erythematosus patients ( n = 24/30, 80%) and healthy controls ( n = 33/39, 84.6%). There was no increase in median Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2K scores and anti-ds DNA levels after the vaccination procedure. Seroconversion rates in childhood-onset systemic lupus erythematosus patients were not affected by medication, high disease activity (SLEDAI-2K >6) and anti-ds DNA positivity. None of the patients experienced any flare or adverse reaction throughout the study. Conclusions: According to these results, we conclude that inactivated hepatitis A vaccine is safe and well tolerated in childhood-onset systemic lupus erythematosus patients, with no adverse events or increase in activity. Immunogenicity to the hepatitis A vaccine was adequate, with a seropositivity rate of 80%.

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 Th cytokine profile in childhood-onset systemic lupus erythematosus

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wei Quan ◽  
Jingnan An ◽  
Gang Li ◽  
Guanghui Qian ◽  
Meifang Jin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Healthy Control Group ◽  
Control Group ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Healthy Control ◽  
Ifn Γ ◽  
Onset Systemic Lupus Erythematosus

Abstract Background Childhood-onset systemic lupus erythematosus (cSLE) is a kind of chronic inflammatory disease characterized by a highly abnormal immune system. This study aimed to detect the serum levels of Th (T helper) cytokines (IL-2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-13, IL-17A, IL-17F, IL-21, IL-22, IFN-γ and TNF-α) in cSLE and healthy controls, and then to elucidate their association with clinical manifestations, disease activity and laboratory parameters. In order to provide clues for early diagnosis and timely intervention treatment of cSLE patients. Methods A total of 33 children with cSLE and 30 healthy children were enrolled in this study. Children in the cSLE group were classified into the inactive or active cSLE group according to their SLE disease activity index 2000 (SLEDAI-2 K) score. Th cytokine profiles in the peripheral blood were detected and analysed. Results Levels of IL-2, IL-10 and IL-21 in the cSLE group were significantly higher than those in the healthy control group (P < 0.05, P < 0.01 and P < 0.01, respectively). Expression of IL-2, IL-10 and IL-21 in the active cSLE group was significantly higher than that in the healthy control group (P < 0.05, P < 0.01 and P < 0.05, respectively), but that of IL-22 expression was markedly lower in the active cSLE group than in the healthy control group (P < 0.001). IL-21 in the inactive SLE group was significantly higher than that in the healthy control group (P < 0.05), and levels of IL-2 and IL-10 in the active cSLE group were significantly higher than those in the inactive cSLE group (P < 0.01 and P < 0.05). In-depth analysis showed that after excluding age, gender and drug interference, the levels of IL-2 (P < 0.05), IL-6 (P < 0.05) and IL-10 (P < 0.05) were still positively correlated with SLEDAI-2 K scores. However, the levels of IL-6 (P < 0.05) and IFN- γ (P < 0.05) were still negatively correlated with CD4+/CD8+, and the concentration of IL-6 (P < 0.05) was still positively correlated with the occurrence of nephritis. Conclusion This study provides a theoretical basis for the discovery of effective methods to regulate imbalance in T lymphocyte subsets in cSLE, which may lead to new approaches for the diagnosis of cSLE.

scholarly journals Evaluation of Hydroxychloroquine Blood Concentrations and Effects in Childhood-Onset Systemic Lupus Erythematosus

2021 ◽  
Vol 14 (3) ◽  
pp. 273
Author(s):  
Noël Zahr ◽  
Saik Urien ◽  
Christian Funck-Brentano ◽  
Hélène Vantomme ◽  
Nicolas Garcelon ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Blood Concentration ◽  
Disease Status ◽  
Concentration Effect ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Blood Concentrations ◽  
Onset Systemic Lupus Erythematosus

Background: Hydroxychloroquine (HCQ) is an antimalarial agent given to patients with systemic lupus erythematosus (SLE) as first-line therapy. It alleviates childhood-onset systemic lupus erythematosus cSLE skin and musculoskeletal disease, decreasing disease activity and flares. HCQ concentration–effect relationships in children remains unknown. This study aimed to investigate the pharmacokinetics of HCQ and possible concentration–effect relationships. Methods: HCQ blood concentrations and effects were obtained during clinical follow-up on different occasions. cSLE flares were defined using the SLE Disease Activity Index (SLEDAI); flare was denoted by a SLEDAI score > 6. Blood concentration was measured using high-performance liquid chromatography with fluorometric detection. Statistical analysis was performed using a nonlinear mixed-effect approach with the Monolix software. Results: A total of 168 blood samples were obtained from 55 pediatric patients. HCQ apparent blood clearance (CL/F) was dependent on patients’ bodyweight and platelet count. Patients with active cSLE had a lower mean blood HCQ concentration compared with inactive cSLE patients (536 ± 294 vs. 758 ± 490 ng/mL, p = 5 × 10−6). Among patients with HCQ blood concentration ≥750 ng/mL, 87.6% had inactive cSLE. Moreover, HCQ blood concentration was a significant predictor of disease status. Conclusion: We developed the first HCQ blood concentration–effect relationship for cSLE associated with active or non-active disease status. A prospective randomized study is necessary to confirm these results.

scholarly journals Childhood-Onset Systemic Lupus Erythematosus: Southeast Asian Perspectives

2021 ◽  
Vol 10 (4) ◽  
pp. 559
Author(s):  
Swee Ping Tang ◽  
Sern Chin Lim ◽  
Thaschawee Arkachaisri
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Economic Status ◽  
Healthcare Access ◽  
Organ Damage ◽  
Southeast Asian ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Childhood onset systemic lupus erythematosus is a rare disease that is more common amongst Southeast Asian children compared to the West. It is typified by a peripubertal onset and a female preponderance, which increases with advancing age. Organs commonly involved at diagnosis include haematological, renal, and mucocutaneous. Fever, malar rash, and cutaneous vasculitis are common. Lupus nephritis is typically proliferative especially Class IV and contributes to both disease activity and damage. Antinuclear antibody and anti-dsDNA positivity are both prevalent in this region. Disease activity is higher than Western cohorts at onset but responds to therapy reducing to low disease activity by six months. However, organ damage occurs early and continues to accumulate over the time, a consequence of both active disease (neurological and renal systems) and steroid-related complications especially in the eye (cataract and glaucoma) and musculoskeletal systems (avascular necrosis). Infections remain the leading cause of death and mortality in this region is highly variable contributed by the heterogeneity in social economic status, healthcare access, and availability of paediatric rheumatology expertise in the region.

Safety and Efficacy of Rituximab in Childhood-onset Systemic Lupus Erythematosus and Other Rheumatic Diseases

2015 ◽  
Vol 42 (3) ◽  
pp. 541-546 ◽  
Author(s):  
Ajay Tambralli ◽  
Timothy Beukelman ◽  
Randy Quentin Cron ◽  
Matthew Laurence Stoll
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Safety Data ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Safety And Efficacy ◽  
Autoimmune Conditions ◽  
Effectiveness Data ◽  
Onset Systemic Lupus Erythematosus

Objective.Rituximab (RTX) has been used to treat many pediatric autoimmune conditions. We investigated the safety and efficacy of RTX in a variety of pediatric autoimmune diseases, especially systemic lupus erythematosus (SLE).Methods.Retrospective study of children treated with RTX. Effectiveness data was recorded for patients with at least 12 months of followup; safety data was recorded for all subjects.Results.The study included 104 children; 50 had SLE. Improvements in corticosteroid dosage, physician’s global assessment of disease activity, and SLE-associated markers of disease activity were seen. The incidence of hospitalized infections was similar to previous studies of patients with childhood-onset SLE.Conclusion.RTX can be safely administered to children and appears to contribute to decreased disease activity and steroid burden.

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