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Introduction: Systemic lupus erythematosus (SLE), the commonest type of lupus, is an autoimmune multisystemic disorder that can affect any organ system of the body, especially blood vessels and connective tissues, causing widespread inflammation. Pediatric onset of SLE is a rare condition with more hematological involvement. Aim: This study was undertaken to observe various hematological abnormalities and their association with various autoantibodies present in pediatric SLE in Eastern India. Methodology: It was a single-centered, cross-sectional, observational, hospital-based study conducted in the Department of Pediatric Medicine in collaboration with the Department of Rheumatology in IPGME and R and SSKM Hospital, Kolkata. The duration of the study was 1.5 years, and a total of 30 children up to 12 years of age of either gender were enrolled. Study participants were evaluated for various parameters like demographic, hematological (anemia, neutropenia, leucopenia, lymphopenia, and thrombocytopenia), biochemical (CRP, Lactate dehydrogenase (LDH), and bilirubin), autoantibodies (anti-dsDNA, anti-Ro 52, and anti-Ribonucleoprotein [RNP]), and SLE related pathologies (Cutaneous, nephritis, serositis). Results: In the present study, most of the participants had arthritis, muscle pain (86.66%), and hematological involvement (80%). Among cytopenias, anemia was the commonest. dsDNA autoantibody was positive in most of the patients (83%), and about one-third suffered from autoimmune hemolytic anemia (AIHA). No association was observed between autoantibodies and various hematological manifestations. Conclusion: It can be concluded from the present study that anemia is the most common cytopenia in pediatric SLE, but there is no association between autoantibodies and these cytopenias. However, study on larger population may give better results.

Abstract Background Systemic lupus erythematosus (SLE) is a life-threatening, chronic, autoimmune disease requiring long term subspecialty care due to its complex and chronic nature. Childhood-onset SLE (cSLE) is more severe than adult-onset, and the cSLE population in South Africa has been reported to have an even higher risk than patients elsewhere. Therefore, it is critical to promptly diagnose, treat, and manage cSLE. In this paper, we aim to describe and evaluate barriers and enablers of appropriate long-term care of cSLE South Africa from the perspective of caregivers (parents or family members). Methods Caregivers (n = 22) were recruited through pediatric and adult rheumatology clinics. Individuals were eligible if they cared for youth (≤ 19 years) who were diagnosed with cSLE and satisfied at least four of the eleven ACR SLE classification criteria. Individual in-depth, semi-structured interviews were conducted between January 2014 and December 2014, and explored barriers to and facilitators of ongoing chronic care for cSLE. Data were analyzed using applied thematic analysis. Results Four barriers to chronic care engagement and retention were identified: knowledge gap, financial burdens, social stigma of SLE, and complexity of the South African medical system. Additionally, we found three facilitators: patient and caregiver education, robust support system for the caregiver, and financial support for the caregiver and patient. Conclusion These findings highlight multiple, intersecting barriers to routine longitudinal care for cSLE in South Africa and suggest there might be a group of diagnosed children who don’t receive follow-up care and are subject to loss to follow-up. cSLE requires ongoing treatment and care; thus, the different barriers may interact and compound over time with each follow-up visit. South African cSLE patients are at high risk for poor outcomes. South African care teams should work to overcome these barriers and place attention on the facilitators to improve care retention for these patients and create a model for other less resourced settings.

Background: Systemic lupus erythematosus (SLE) is a life-threatening, chronic, autoimmune disease requiring long term subspecialty care due to its complex and chronic nature. Childhood-onset SLE (cSLE) is more severe than adult-onset, and the cSLE population in South Africa has been reported to have an even higher risk than patients elsewhere. Therefore, it is critical to promptly diagnose, treat, and manage cSLE. In this paper, we aim to describe and evaluate barriers and enablers of appropriate long-term care of cSLE South Africa from the perspective of caregivers (parents or family members). Methods: Caregivers (n = 22) were recruited through pediatric and adult rheumatology clinics. Individuals were eligible if they cared for youth (≤ 19 years) who were diagnosed with cSLE and satisfied at least four of the eleven ACR SLE classification criteria. Individual in-depth, semi-structured interviews were conducted between January 2014 and December 2014, and explored barriers to and facilitators of ongoing chronic care for cSLE. Data were analyzed using applied thematic analysis. Results: Four barriers to chronic care engagement and retention were identified: knowledge gap, financial burdens, social stigma of SLE, and complexity of the South African medical system. Additionally, we found three facilitators: patient and caregiver education, robust support system for the caregiver, and financial support for the caregiver and patient. Conclusion: These findings highlight multiple, intersecting barriers to routine longitudinal care for cSLE in South Africa and suggest there might be a group of diagnosed children who don't receive follow-up care and are subject to loss to follow-up. cSLE requires ongoing treatment and care; thus, the different barriers may interact and compound over time with each follow-up visit. South African cSLE patients are at high risk for poor outcomes. South African care teams should work to overcome these barriers and place attention on the facilitators to improve care retention for these patients and create a model for other less resourced settings. [ABSTRACT FROM AUTHOR]

Introduction: Systemic lupus erythematosus (SLE), the commonest type of lupus, is an autoimmune multisystemic disorder that can affect any organ system of the body, especially blood vessels and connective tissues, causing widespread inflammation. Pediatric onset of SLE is a rare condition with more hematological involvement. Aim: This study was undertaken to observe various hematological abnormalities and their association with various autoantibodies present in pediatric SLE in Eastern India. Methodology: It was a single‑centered, cross‑sectional, observational, hospital‑based study conducted in the Department of Pediatric Medicine in collaboration with the Department of Rheumatology in IPGME and R and SSKM Hospital, Kolkata. The duration of the study was 1.5 years, and a total of 30 children up to 12 years of age of either gender were enrolled. Study participants were evaluated for various parameters like demographic, hematological (anemia, neutropenia, leucopenia, lymphopenia, and thrombocytopenia), biochemical (CRP, Lactate dehydrogenase (LDH), and bilirubin), autoantibodies (anti‑dsDNA, anti‑Ro 52, and anti‑Ribonucleoprotein [RNP]), and SLE related pathologies (Cutaneous, nephritis, serositis). Results: In the present study, most of the participants had arthritis, muscle pain (86.66%), and hematological involvement (80%). Among cytopenias, anemia was the commonest. dsDNA autoantibody was positive in most of the patients (83%), and about one‑third suffered from autoimmune hemolytic anemia (AIHA). No association was observed between autoantibodies and various hematological manifestations. Conclusion: It can be concluded from the present study that anemia is the most common cytopenia in pediatric SLE, but there is no association between autoantibodies and these cytopenias. However, study on larger population may give better results. [ABSTRACT FROM AUTHOR]

Infections are a major cause of morbidity and mortality in juvenile systemic lupus erythematosus (SLE). We assessed the incidence and risk factors for major infections in juvenile SLE.A retrospective review of 225 patients of juvenile SLE (ACR 1997 criteria) with age <18 years visiting the rheumatology clinic at a single centre between 2000 to 2020 was done from case records and the hospital electronic health records. Serious infection was defined as the need for hospitalization, or infection resulting in disability or death. Cox regression was used to determine factors associated with a serious infection and the effect of serious infection on overall survival.We reviewed 225 children (197 girls, mean age 13.89 ± 3.42 years) with a cumulative follow up of 1153.45 person-years. Eighty serious infections occurred in 63 (28% of the cohort) children at a rate of 69.35 serious infections per 1000 person-years. A second serious infection occurred in 12 children and 5 of them developed three infections.Among the cases with known etiology (78.75% of cases), bacterial infections were most common (N = 33) including S. Aureus (11), E. Coli (7), K. Pneumoniae (3), E. Fecalis (3), S. Pneumoniae (2), Acinetobacter spp. (2), Citrobacter (2), Salmonella (2) and P. Aeruginosa (1). Twenty six (32.5%) opportunistic infections occurred: Mycobacterium tuberculosis (18), Cytomegalovirus (3), disseminated Herpes zoster (4) and invasive candidiasis (1) with 15 (83.3%) of the tuberculosis cases being extrapulmonary. On multivariate analysis, fever (HR 8.51, 1.17–61.44), gastrointestinal involvement (HR 4.73, 1.13–19.94), current steroid dose (HR 1.36,1.14–1.62), average cumulative steroid dose per year (HR 1.004, 1.002–1.005) and cyclophosphamide (HR 2.22, 1.11–4.46) were associated with serious infection.Hospitalization rates were significantly higher in those with any serious infection (Rate-ratio 2.79, 1.81–3.77) as was damage accrual (SLICC damage index 1.04 vs 0.22). Serious infection-free survival at 1 year and 5 years was 84% (79.1–89.2) and 72% (65.4–79.2). There were 19 deaths with infection attributable mortality in 10 (52.6%). Serious infection predisposed to higher overall mortality with recurrent infections conferring a hazard ratio of 36.02 (8.07–160.62).Serious infections are a major cause of mortality and damage in SLE. Constitutional symptoms, gastrointestinal involvement, current and cumulative steroid dose and cyclophosphamide predict serious infections. TB prophylaxis in patients with SLE should be considered in endemic areas, especially when using high-dose steroid therapy. [ABSTRACT FROM AUTHOR]

Introduction: Childhood systemic lupus erythematosus (cSLE) is a complex multisystem autoimmune disease. In 2019, belimumab was approved for the clinical treatment for cSLE, making it the only biological agent approved for cSLE children aged 5 and older in 60 years. Objective: To review emerging evidence on belimumab in cSLE published up to April 2022, so as to provide information for clinical decision-making. Method: A comprehensive search of relevant publications up to the date of April 2022 in PUBMED, EMBASE, WOS, COCHRANE, ClinicalTrials.gov, CBM, CNKI and WANFANG was performed using the following criteria: (a) English and Chinese language studies; (b) RCT studies, cohort studies, or case-control studies; (c) patients with age <18; (d) Observational studies or case series studies contain more than 5 patients. All relevant literature was independently screened and reviewed by at least two reviewers and the obtained literature data were extracted and reviewed by two authors. Results: Five publications met the inclusion/exclusion criteria for cSLE: one randomized controlled trial, one retrospective cohort study, and three case series. There was a high degree of heterogeneity among several studies, and the availability of baseline and outcome data provided was uneven. Conclusion: At present, there is a lack of high-quality clinical trials of belimumab in the treatment of cSLE. Based on the current research, it is believed that the use of belimumab can inhibit cSLE activity, reduce the dose of corticosteroids and immunosuppressants, and delay kidney damage. Also it shows clinical benefit in alleviating symptoms of monogenic cSLE refractory to standard therapy. More studies are urgently needed to validate the clinical efficacy of belimumab in cSLE and to evaluate its long-term safety in pediatric populations to promote evidence-based practice. [ABSTRACT FROM AUTHOR]

Objective: The objective is to determine the 30-day hospital readmission rate following a hospitalization due to pediatric lupus nephritis of recent onset and characterize the risk factors associated with these early readmissions. Methods: The study included 76 children hospitalized from 01/01/2008 to 4/30/2017 due to a new diagnosis of lupus nephritis. We calculated the 30-day hospital readmission rate and compared the characteristics of the patients that were readmitted to patients that were not readmitted using univariable and multivariable analysis. Results: The 30-day readmission rate was 17.1%. Factors that predicted hospital readmission in unavailable analysis were male gender (38.5 vs 14.3%, p = 0.04), not receiving pulse steroids (30.8 vs 3.2%, p = <.001), receiving diuretic treatment (69.2 vs 34.9%, p =.02), receiving albumin infusions (46.2 vs 12.7%, p =.004), stage 2 hypertension on day one of admission (76.9 vs 41.3%, p =.02), a higher white blood cell count on discharge (13.7 × 103/mm3 vs 8.8 × 103/mm 3 , p =.023), need for non-angiotensin converting enzyme (ACE) antihypertensive drugs (76.9 vs 46%, p =.042), and being discharged on nonsteroidal anti-inflammatory drugs (NSAIDs) (23.1 vs 4.8%, p =.025). Multivariable analysis demonstrated an increased risk of readmission for patients not treated with intravenous pulse methylprednisolone (IVMP) (OR = 17.5 (1.81–168.32) p =.013), and for those who required intravenous albumin assisted diuresis for hypervolemia (OR=6.25 (1.29–30.30) p =.022). Conclusion: In all, 17% of children hospitalized due to new onset lupus nephritis were readmitted within 30 days of discharge. Absence of IVMP and receiving intravenous albumin assisted diuresis during initial hospitalization increase the risk of early readmission in new onset pediatric lupus nephritis. [ABSTRACT FROM AUTHOR]

Introduction: Children with systemic lupus erythematosus have a more challenging and difficult course as compared to their adult counterparts. Today, the aim of therapy for any child with lupus is to keep the child in a state of sustained remission with minimal or no use of steroids. This laudable goal is often difficult to achieve for the child with lupus. In addition to the use of disease modifying agents, sometimes in combination, Rituximab (RTX) is also used as an off-label indication to manage such patients. Objectives: To study the use, efficacy and safety of RTX in a cohort of patients with pediatric lupus followed at a single tertiary level center in Northern India. Methods: This paper is a retrospective review looking at the use of RTX in children with systemic lupus at a tertiary level pediatric rheumatology center in North India over a period of seventeen years. This paper describes the indications, use, efficacy and safety of RTX in childhood systemic lupus erythematosus. Results: RTX was used in 17 of 225 pediatric lupus patients (7.5%), with the most common indication being resistant renal disease (53%). Significant improvement was seen in all domains studied: The mean SLEDAI was 16.25 prior to RTX and reduced to 1.43 six months after the RTX (p value 0.001), steroid use dropped from 100% pre- RTX to 33% at 2 years, there was a sustained reduction in proteinuria in the patients with nephritis from a mean urine spot protein creatinine ratio of 3.1 pre RTX to 0.4 at one year post RTX (p=.006). Finally, 82% of the children had no flare during the follow up (median 24 months). No patient had any adverse event. Conclusions: This study confirms that RTX is very effective in childhood lupus and can be safely used even in a country with a very high burden of infectious diseases. This data adds to the scarce literature in this area from the developing world. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the reliability, validity, feasibility and psychometric performance of the Lupus Impact Tracker (LIT) as a patient reported outcome (PRO) measure tool in pediatric systemic lupus erythematosus (pSLE). Methods: This is a prospective, observational, pilot study where patients aged between 12 and 25 years, fulfilling the 1997 ACR classification criteria for SLE, were enrolled. Over 3 consecutive, routine, clinical visits, the patients completed the LIT alongside the Patient-Reported Outcomes Measurement Information System-Short Forms (PROMIS-SFs), Childhood Health Assessment Questionnaire (CHAQ). Rheumatologists completed the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC-ACR) Damage Index. Demographic, clinical and laboratory data were also collected. Results: Of 46 patients enrolled, 38 patients completed 2 visits and 31 completed all 3 visits. Seventy-eight percent were female, 33% African American, 28% Asian, 15% Caucasian and 17% Hispanic. The mean (SD) age was 17.2 (2.7) years, with a mean (SD) disease duration of 4.6 (3.1) years. The mean (SD) SLEDAI-2K at enrollment was 3.54 (2.96). In the 38 patients who completed two or more visits, intra-class correlation coefficient and Cronbach alpha were calculated to be 0.70 and 0.91 respectively, signifying good reliability of LIT. The LIT showed positive correlation with CHAQ-Disability Index and majority of the PROMIS-SFs parameters. Construct validity was established against clinical disease activity (SLEDAI-2K). Conclusion: The preliminary results indicate that the LIT is a reliable and valid instrument to capture PRO in p-SLE. Prospective validation with a larger, multicenter cohort is the next step. [ABSTRACT FROM AUTHOR]

The increased relative risk of heart failure (HF) from systemic lupus erythematosus (SLE) is greatest at younger ages, but the etiology remains unclear. We identified risk factors for HF in children and adults with SLE and evaluated associations between SLE manifestations and HF. Incident SLE cases without preceding HF were identified using Clinformatics DataMart® (OptumInsight, Eden Prairie, MN) US claims data (2000-2015), and categorized by age of SLE onset (children 5–17, young adults 18–24, adults 25–44 years old). The primary outcome was the first HF ICD-9-CM diagnosis code (428.x), categorized as early-onset (< 6 months) or delayed-onset. Multivariable logistic regression was used to identify factors associated with early or delayed-onset HF. Cox proportional hazards regression was used to identify time-dependent associations between the onset of SLE manifestations and incident HF. There were 523 (2.3%) HF cases among 1,466 children, 2,163 young adults and 19,349 adults age 25–44 with SLE. HF in children and young adults was early-onset in 50% and 60% of cases, respectively, compared to 35% of cases in adults 25–44 years old. There was a temporal association between incident myopericarditis and valvular disease diagnoses and early-onset HF, whereas nephritis and hypertension were more strongly associated with delayed-onset HF. Black race remained independently associated with a 1.5-fold increased HF risk at any time. Hypertension remains an important traditional CV risk factor across all ages and should be managed aggressively even in younger patients with SLE. Cardiac dysfunction due to acute cardiac manifestations of SLE may contribute to the very high relative incidence of early HF diagnoses among younger SLE patients. Therefore, future prospective studies will need to address heterogeneity in the types and severity of heart failure in order to determine etiology and which patients should be monitored. [ABSTRACT FROM AUTHOR]

The pathophysiology of systemic lupus erythematosus (SLE) has been intensely studied but remains incompletely defined. Currently, multiple mechanisms are known to contribute to the development of SLE. These include inadequate clearance of apoptotic debris, aberrant presentation of self nucleic antigens, loss of tolerance, and inappropriate activation of T and B cells. Genetic, hormonal, and environmental influences are also known to play a role. The study of lupus in children, in whom there is presumed to be greater genetic influence, has led to new understandings that are applicable to SLE pathophysiology as a whole. In particular, characterization of inherited disorders associated with excessive type I interferon production has elucidated specific mechanisms by which interferon is induced in SLE. In this review, we discuss several monogenic forms of lupus presenting in childhood and also review recent insights gained from cytokine and autoantibody profiling of pediatric SLE.

Pneumococcal vaccination is recommended as a quality indicator for management of children with systemic lupus erythematosus. Literature on the immunogenicity of pneumococcal vaccines (PCVs) in children is scant. We sought to prospectively evaluate via an observational study, the immunogenicity to sequential children with lupus. Out of a cohort of 26 patients, approximately 65% achieved > 70% vaccinated serotype antibody levels of > 1.3 mcg/dL following PCV13, and of 22 patients followed through PPSV23 vaccination, 59% achieved the same. Patients with rituximab exposure in the 6 months prior to a vaccination were more likely to not achieve protective serotype levels (p < 0.01 for PCV13, trend p = 0.07 for PPSV23). Three of 22 patients with no apparent risk factors did not achieve protective serotype levels. Non-responders to PCV13 generally did not respond to PPSV23. Retrospective healthy controls achieved 100% protective levels in response to PPSV23 vaccination, with 95% of serotypes being > 1.3 mcg/dL. Thus, sequential 13- and 23-valent pneumococcal vaccines achieve protective status for approximately two thirds of pediatric lupus patients in our population. Lack of response to vaccine may be secondary to induced or inherent functional impairments in the patient. [ABSTRACT FROM AUTHOR]

Introduction About 10–20% of systemic lupus erythematosus (SLE) patients have onset in childhood and have more severe organ involvement. Survival of juvenile SLE patients is improving worldwide. Long-term data of childhood onset SLE from developing countries is scarce. Methods Clinical and laboratory data at initial presentation and follow-up visits were retrieved from clinic files, hospital information system and personal interviews. Treatment received, complications, flares, outcomes and death were recorded. Survival was calculated using Kaplan–Meier survival curves and regression analysis was done for predictors of mortality. Results Children with SLE (n = 273, 250 girls) had a median age at onset of 14 years and duration of illness prior to diagnosis at our hospital of 1 year. Fever and arthritis were the most common presenting manifestations. Renal disease was seen in 60.5% and central nervous system (CNS) disease in 29%. The median follow-up period in 248 patients was 3.5 years. Fourteen children died, and 10 of these had active disease at the time of death. The mean actuarial survival was 24.5 years and survival rates at 1, 5 and 10 years were 97.9%, 95% and 89% respectively. Fever, CNS disease, anti-dsDNA levels and serious infections predicted death on univariate and multivariate analysis. Infections were seen in 72 children (26.3%), and 38 of these infections were serious. One-third of the patients had damage on the last follow-up. Flares were seen in 120 children, the majority being major flares. Conclusion Outcomes of pediatric SLE in North Indian children are similar to those seen in developed countries. Infections pose a major challenge in these patients. [ABSTRACT FROM AUTHOR]

Background Systemic lupus erythematosus (SLE) is a life-threatening multisystem autoimmune disease that is more severe in patients of African ancestry and children, yet pediatric SLE on the African continent has been understudied. This study describes a cohort of pediatric SLE (PULSE) patients in South Africa. Methods Patients with a diagnosis of SLE (1997 American College of Rheumatology criteria) diagnosed prior to age 19 years in Cape Town, South Africa, were enrolled in this cross-sectional study from September 2013 to December 2014. Information on clinical and serological characteristics was extracted from medical records. Results were compared to a well-described North American pediatric SLE cohort. Results Seventy-two South African patients were enrolled in the study; mean age 11.5 years; 82% were girls. The racial distribution was 68% Coloured, 24% Black, 5% White and 3% Asian/Indian. Most patients presented with severe lupus nephritis documented by renal biopsy (61%). Of patients with lupus nephritis, 63% presented with International Society of Nephrology/Renal Pathology Society class III or IV. Patients in the PULSE cohort were more likely to be treated with cyclophosphamide, methotrexate and azathioprine. The PULSE cohort had high disease activity at diagnosis (mean Systemic Lupus Erythematosus Disease Activity Index-2K (SLEDAI-2K) 20.6). The SLEDAI-2K at enrolment in the PULSE cohort (5.0) did not differ from the North American pediatric SLE cohort (4.8). Sixty-three per cent of the PULSE cohort had end organ damage with Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI) score >0 (mean SLICC-DI 1.9), compared to 23% in a previously reported US cohort. Within the PULSE cohort, nine (13%) developed end-stage renal disease with six (8%) requiring transplant, strikingly higher than North American peers (transplant rate <1%). Conclusions The PULSE cohort had highly active multiorgan disease at diagnosis and significant disease damage at enrolment in the South African registry. South African patients have severe lupus nephritis and poor renal outcomes compared to North American peers. Our study revealed a severe disease phenotype in the PULSE cohort resulting in poor outcomes in this high-risk population. [ABSTRACT FROM AUTHOR]

Lupus nephritis is one of the most common and serious complications of systemic lupus erythematosus (SLE) in childhood affecting more than 80 % of patients. Treatment of this complication has undergone significant evolution in recent years. A series of randomized controlled trials has clarified the role of a variety of immunomodulating regimens including some novel biologic medications. This review touches on the major trials that have influenced practice and shaped current thinking about the treatment of proliferative lupus glomerulonephritis. [ABSTRACT FROM AUTHOR]

Children with systemic lupus erythematosus (SLE) have a high prevalence of antiphospholipid (aPL) antibodies and are at increased risk for aPL-related thrombosis. We investigated the association between annexin A5 anticoagulant activity and antibodies to the domain I portion of β2-glycoprotein I (anti-DI antibodies), and propose a potential mechanism for the pathogenesis of aPL-related thrombosis. Using samples from 183 children with SLE collected during the Atherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) trial, we examined resistance to the anticoagulant effects of annexin A5, using the annexin A5 resistance (A5R) assay, and evaluated for anti-DI IgG antibodies. Children with SLE had higher frequency of anti-D1 antibodies (p = 0.014) and significantly reduced A5R compared to pediatric controls: mean A5R = 172 ± 30% versus 242 ± 32% (p < 0.0001). Children with SLE and positive anti-DI antibodies had significantly lower mean A5R levels compared to those with negative anti-DI antibodies: mean A5R = 155 ± 24% versus 177 ± 30% (p < 0.0001). In multivariate analysis, anti-DI antibodies (p = 0.013) and lupus anticoagulant (LA) (p = 0.036) were both independently associated with reduced A5R. Children with SLE have significantly reduced annexin A5 anticoagulant activity that is associated with the presence of LA and anti-DI antibodies. [ABSTRACT FROM AUTHOR]

Introduction: Childhood systemic lupus erythematosus (cSLE) is a complex multisystem autoimmune disease. In 2019, belimumab was approved for the clinical treatment for cSLE, making it the only biological agent approved for cSLE children aged 5 and older in 60 years., Objective: To review emerging evidence on belimumab in cSLE published up to April 2022, so as to provide information for clinical decision-making., Method: A comprehensive search of relevant publications up to the date of April 2022 in PUBMED, EMBASE, WOS, COCHRANE, ClinicalTrials.gov, CBM, CNKI and WANFANG was performed using the following criteria: (a) English and Chinese language studies; (b) RCT studies, cohort studies, or case-control studies; (c) patients with age <18; (d) Observational studies or case series studies contain more than 5 patients. All relevant literature was independently screened and reviewed by at least two reviewers and the obtained literature data were extracted and reviewed by two authors., Results: Five publications met the inclusion/exclusion criteria for cSLE: one randomized controlled trial, one retrospective cohort study, and three case series. There was a high degree of heterogeneity among several studies, and the availability of baseline and outcome data provided was uneven., Conclusion: At present, there is a lack of high-quality clinical trials of belimumab in the treatment of cSLE. Based on the current research, it is believed that the use of belimumab can inhibit cSLE activity, reduce the dose of corticosteroids and immunosuppressants, and delay kidney damage. Also it shows clinical benefit in alleviating symptoms of monogenic cSLE refractory to standard therapy. More studies are urgently needed to validate the clinical efficacy of belimumab in cSLE and to evaluate its long-term safety in pediatric populations to promote evidence-based practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Chen, Zheng, Chen, Wen, Xu, Yang and Xu.)

Abstract: Systemic lupus erythematosus is a multisystemic, autoimmune disorder, with varied clinical and immunological manifestations. Though the predominant onset is in young adulthood especially in females, childhood-onset, with a much broader spectrum of clinical presentations, has been reported with incidence rates ranging from 0.36 to 2.5 per 100,000. Diverse immune system aberrations, both inherited and acquired, contribute to the organ damage. Lupus nephritis is seen in a high proportion of childhood-onset disease and is an important contributor for both short-term and long-term morbidity. Various immunosuppressive regimes with a common goal of achieving and maintaining renal remission have been tested mainly in the adult population. The management of childhood-onset lupus nephritis based on the current available evidence is reviewed and the most appropriate management approach for this group of patients is discussed. [Copyright &y& Elsevier]

The objective is to study the neuropsychiatric (NP) manifestations in pediatric onset systemic lupus erythematosus (SLE) at a tertiary care hospital of northwestern India applying American College of Rheumatology (ACR) case definitions in the context of occurrence of antiphospholipid antibodies (APLA). Data of 53 children with SLE were analyzed for NP syndromes. Tests for detection of APLA were performed as per international standards for quality control. Twenty-seven of the 53 (50.94%) children with lupus had at least one NP manifestation. The male to female ratio of our cohort of pediatric lupus was 1:2.8. However, there was significant male preponderance in patients with NPSLE as compared to patients without NPSLE (1:1.25 vs. 1:12; P < 0.0001). Majority of children with NPSLE (15/27, 55.5%) already had NP manifestations at the time of diagnosis and most of them (81.5%) had experienced more than one NP symptom. Headache was the commonest NP manifestation and was seen in 39.6% children with SLE followed by seizure disorder (35.8%) and cognitive dysfunction (16.9%). Tests for APLA were carried out in 37 of 53 (69.8%) patients with SLE and in 24 of 27 (88.8%) patients with NPSLE. While anticardiolipin antibodies were seen more frequently in children with NPSLE as compared to those without NPSLE (57.8 vs. 23%), lupus anticoagulant was more frequent in children without NPSLE (53.8 vs. 34.7%). However, these differences were statistically not significant. Eleven of the 27 children with NPSLE succumbed to their illness, primarily due to uncontrolled disease activity. Mean duration of follow-up of patients with NPSLE who are alive was 65.4 ± 36.9 months. NP manifestations are common in pediatric onset lupus and contribute to significant morbidity. As compared to previously published literature, a significantly greater proportion of boys were affected. APLA were frequently detected in children with NPSLE. There is paucity of literature pertaining to NP manifestations of pediatric lupus in the context of APLA, especially with regard to antibodies to beta-2 glycoprotein I. To the best of our knowledge, this is the first detailed study on NP manifestations in childhood lupus from a developing country applying ACR case definitions. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease characterized by periods of increased disease activity caused by inflammation of blood vessels and connective tissue. Pediatric patients with SLE have a more severe clinical course when compared with adults. Patients commonly present with rash, fever, and arthritis, although the presentation may be unpredictable. Hematological findings are more predominant in children than adults. Thirty-nine percent of children with SLE will develop hematological abnormalities, one of the American Rheumatic Association criteria for classifying the disease. In our case series we found varied hematological picture and presentation. We present here four case reports of SLE cases with interesting hematological features. Our first case is a 13 month old female child who was initially diagnosed as Evans syndrome and 2 years later diagnosed as SLE. Second case is a 3 year old male child who had SLE with warm antibody AIHA. Third case is a 6 year old female child who presented with AIHA and was diagnosed with SLE 6 years later. Fourth case is a 6 year old female child diagnosed as SLE with aplastic anemia. Hematological findings should be carefully assessed and treated in order to decrease disease related morbidity. [ABSTRACT FROM AUTHOR]

Acute septic arthritis in association with systemic lupus Erythematosus (SLE) is uncommon and mostly caused by Gram positive bacteria. Escherichia coli (E coli) septic arthritis complicating lupus is extremely uncommon. The authors describe a case in a 10 year old Indian girl who had SLE with class II lupus nephritis and presented later with acute septic arthritis in her right knee. Although reported earlier in adult subjects, pediatric SLE complicated with E coli septic arthritis at this young age, to the best of authors' knowledge has never been reported in world literature. [ABSTRACT FROM AUTHOR]

Abstract: Neuropsychiatric syndromes are prevalent in pediatric patients with systemic lupus erythematosus (SLE) and often manifest early in disease course and with significant associated morbidity. Postulated pathogenic mechanisms of peripheral and central nervous system events include vasculopathy, autoantibody effects and systemic inflammation. The pathogenic roles of anti-phospholipid, anti-ribosomal-P and anti-neuronal autoantibodies have been examined in both focal and diffuse adult neuropsychiatric syndromes. Few studies have probed associations between these autoantibodies and pediatric neuropsychiatric SLE (NP-SLE). Retrospective review of a large ethnically diverse pediatric SLE cohort revealed anti-phospholipid, anti-ribosomal P, and anti-neuronal antibodies to be more prevalent than in many adult studies. Rates of anti-phospholipid and anti-ribosomal P antibody positivity were similar to those of other pediatric reports. Association between anti-neuronal antibodies and NP-SLE events appeared statistically significant in this cohort. Prospective inception cohort studies will need to be undertaken to investigate the significance and utility of autoantibody testing in pediatric NP-SLE. [Copyright &y& Elsevier]

The pathophysiology of systemic lupus erythematosus (SLE) has been intensely studied but remains incompletely defined. Currently, multiple mechanisms are known to contribute to the development of SLE. These include inadequate clearance of apoptotic debris, aberrant presentation of self nucleic antigens, loss of tolerance, and inappropriate activation of T and B cells. Genetic, hormonal, and environmental influences are also known to play a role. The study of lupus in children, in whom there is presumed to be greater genetic influence, has led to new understandings that are applicable to SLE pathophysiology as a whole. In particular, characterization of inherited disorders associated with excessive type I interferon production has elucidated specific mechanisms by which interferon is induced in SLE. In this review, we discuss several monogenic forms of lupus presenting in childhood and also review recent insights gained from cytokine and autoantibody profiling of pediatric SLE.