Systemic Lupus Erythematosus Children Research Articles

Characteristics and clustering analysis of peripheral blood lymphocyte subsets in children with systemic lupus erythematosus complicated with clinical infection.

Clinical infection is a common complication in children with systemic lupus erythematosus (SLE). However, few studies have investigated immune alterations in children with SLE complicated with clinical infection. We assessed lymphocyte subsets in children with SLE to explore the possibility of clinical infection. We retrospectively analyzed the proportion of peripheral lymphocyte subsets in 140 children with SLE. Children with SLE were classified into different clusters according to the proportion of peripheral blood lymphocyte subsets: (CD3 + /CD4 + T cell, CD3 + /CD8 + T cell, CD3 + /CD4 + /CD8 + T cell, CD3 + /CD4-/CD8- T cell, CD19 + B cell, and CD3-/CD16 + /CD56 + NK cell). Differences in the proportion of lymphoid subsets, infection rates, and systemic lupus erythematosus disease activity index (SLEDAI) scores were compared between clusters. In addition, we grouped the subjects according to the presence or absence of infection. Proportions of lymphoid subsets, demographic variables, clinical presentation, and other laboratory variables were compared between the infected and uninfected groups. Finally, the diagnostic ability of lymphocyte subset ratios to distinguish secondary infection in children with SLE was predicted using an ROC curve. Cluster C2 had a higher proportion of B cells than Cluster C1, while Cluster C1 had a lower proportion of NK cells, CD3 + T cells, CD3 + /CD4 + T cells, CD3 + /CD8 + T cells, and CD3 + /CD4-/CD8- T cells. Infection rates and SLEDAI scores were higher in Cluster C2 than in Cluster C1. The infected children had a higher proportion of B cells and a lower proportion of CD3 + T cells, CD3 + /CD4 + T cells, CD3 + /CD8 + T cells, and CD3 + /CD4-/CD8- T cells. There were no significant differences in lymphoid subsets between children in Cluster C2 and the infected groups. The area under the ROC curve of B lymphocytes in predicting SLE children with infection was 0.842. The area under the ROC curve was 0.855 when a combination of B cells, NK cells, CD4 + T cells, and CD8 + T cells was used to predict the outcome of coinfection. A high percentage of B cells and a low percentage of CD3 + T cells, CD3 + /CD4 + T cells, CD3 + /CD8 + T cells, CD3 + /CD4 + /CD8 + T cells, and CD3 + /CD4-/CD8- T cells may be associated with infection in children with SLE. B cells was used to predict the outcome of coinfection in children with SLE. Key Points •A high percentage of B cells and a low percentage of CD3 + T cells, CD3 + /CD4 + T cells, CD3 + /CD8 + T cells, CD3 + /CD4 + /CD8 + T cells, and CD3 + /CD4-/CD8- T cells may be associated with infection in children with SLE •B cells was used to predict the outcome of coinfection in children with SLE.

Health Related Quality of Life in Juvenile-Onset Systemic Lupus Erythematosus: A Questionnaire-Based Study

ObjectivesThis study aimed to evaluate health related quality of life (HRQOL) in Egyptian children with systemic lupus erythematosus (SLE) using 3 different tools.MethodsIn this questionnaire-based study, 100 children with SLE were included. HRQOL was assessed using the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL™ 4.0 GCS), PedsQL™ 3.0 Rheumatology Module (PedsQL3-RM) and the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY). SLE disease activity index (SLEDAI) was used to evaluate activity and SLE International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) was used to evaluate chronic damage.ResultsAll mean scores of PedsQLTM4.0 GCS domains in SLE patients were lower than published normative data and previously published results of Egyptian healthy controls (p < 0.001). All mean scores of PedsQL-3RM domains were significantly lower than published normative data except for the treatment and pain and hurt domains (p = 0.1, 0.2 respectively). SMILEY scores were low and the lowest domain scores was “Burden of SLE”. Longer duration of illness, higher cumulative steroid doses, higher SLEDAI and SDI scores and presence of obesity were associated with lower scores for all 3 tools (p < 0.001).Conclusion for PracticeThe Arabic copies of PedsQL™ 4.0 GCS, PedsQL3-RM and SMILEY are easily used for Arabic speaking subjects and easily interpreted by physician and can be implemented for frequent monitoring of SLE HRQOL. Controlling the disease activity and using lowest doses of steroids and other immunosuppressive drugs are the corner stone strategies for improving HRQOL in SLE children.

Insulin-like growth factor binding protein-2 as a biomarker for lupus nephritis.

Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus (SLE). The aim of this study was to identify serum insulin-like growth factor binding protein-2 (IGFBP-2) as a novel non-invasive biomarker for clinical disease and renal pathology in pediatric LN. A cross-sectional study on 93 newly diagnosed LN children who were biopsy-proven, 35 SLE children with no renal involvement as disease controls, and 30 healthy controls (HC) with age and gender-matched. All children were ELISA tested for serum IGFBP-2 levels. Clinical, laboratory, histopathological features of LN patients were collected. Compared to SLE or HC, serum IGFBP-2 levels were significantly elevated in LN patients. Serum IGFBP-2 could distinguish LN patients from two others (AUC = 0.937, p < 0.001 for LN vs. HC; 0.897, p < 0.0001 for LN vs. SLE). In ROC analysis, IGFBP-2 had a higher ability to differentiate between LN and SLE than anti-dsDNA with AUC values of 0.895 and 0.643, respectively. LN children with systemic lupus erythematosus disease activity index (SLEDAI) in high activity had significantly higher IGFBP-2 concentration than the others with SLEDAI in moderate activity. Serum IGFBP-2 correlated with albuminemia levels (r = 0.415, p < 0.001), urine protein-to-creatinine levels (r = 0.316, p = 0.002), estimated glomerular filtration rate (r = 0.438, p < 0.001), complement C3 (r = 0.333, p = 0.001). More importantly, serum IGFBP-2 correlated with the activity index of renal pathology (r = 0.312, p = 0.007, n = 75). Serum IGFBP-2 is a promising biomarker for pediatric lupus nephritis, reflective of disease activity and activity index in renal patients.

Assessment of Spirometric Parameters in Systemic Lupus Erythematosus Children with Lupus Nephritis

Assessment of Spirometric Parameters in Systemic Lupus Erythematosus Children with Lupus Nephritis

Exposure levels of mycophenolic acid are associated with comorbidities in children with systemic lupus erythematosus.

Little is known about the relationship between exposure levels of mycophenolic acid (MPA), the active metabolite of mycophenolate mofetil (MMF), and comorbidities of systemic lupus erythematosus (SLE) in children. This study aims to explore this association. Longitudinal data from SLE children, who were taking MMF for immunosuppression and under therapeutic drug monitoring (TDM), were retrospectively collected. Area under the concentration-time curve of mycophenolic acid (MPA) over 24 hours (AUC0-24h) was estimated with Bayesian methods. Logistic regression and random forest models were used to explore the association between comorbidities and MPA exposure levels. This study included 107 children with 358 times of follow-up (median age 169.02 months). The incidence of diabetes, acute kidney injury (AKI), or pneumonia was significantly associated with AUC0-24h (odds ratio [OR] 0.991, 95% confidence interval [CI] 0.982-0.999), SLE duration (OR 1.012, 95% CI 1.002-1.022), lymphocyte percentage (OR 0.959, 95% CI 0.925-0.991), plasma albumin levels (OR 0.891, 95% CI 0.843-0.940), use of aspirin (OR 0.292, 95% CI 0.126-0.633) and hydroxychloroquine (OR 0.407, 95% CI 0.184-0.906). The random forest model showed that albumin and AUC0-24h were two important predictors. The case group (with the three comorbidities) had a mean AUC0-24h of 73.63 mg · h/L, while the control group had a mean AUC0-24h of 100.39 mg · h/L. Increased levels of MPA exposure are associated with decreased incidence odds of diabetes, AKI or pneumonia in SLE children. An AUC0-24h of 100.39 mg · h/L or an AUC0-12h of 50.20 mg · h/L could be used as the targeted exposure level for clinical practice.

Plasma level of deoxyribonuclease I (DNASE I) and its relationship with immune disorders in systemic lupus erythematosus

Background Nucleosomes are the key autoantigens in systemic lupus erythematosus (SLE) patients. Immune complexes including nucleosomes are the chief reason for tissue injury. Deoxyribonuclease 1 (DNASE I), as an endonuclease enzyme, is one of the key molecules involved in the degradation of the apoptosis pathway and its function is diminished in SLE patients.Objective This study aimed to evaluate DNASE I concentration in the plasma of systemic lupus patients and its relationship with immune disorders.Patients and methods This study was carried out on 90 SLE children and 92 healthy controls matched for age and sex. Plasma DNASE I level and levels of serum antinuclear antibody and serum anti-dsDNA were evaluated by the enzyme-linked immunosorbent assay technique. Complement components C3 and C4 were estimated by the turbidimetric assay.Conclusion The authors found a lower level of DNASE I among SLE patients than the healthy control group. In addition, our results suggested no significant association between the enzyme level and immune disorders, or any complications among systemic lupus patients.

Autoimmune haemolytic anaemia and antiphospholipid antibodies in paediatrics: a systematic review and meta-analysis.

The relationship between autoimmune haemolytic anaemia (AIHA) and antiphospholipid antibodies (aPL) has never been addressed via a meta-analysis in the paediatric age group. We evaluated the link between AIHA and aPL in childhood systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). EMBASE and PubMed were screened from inception to May 2020 and Peto's odds ratio for rare events was employed for the between group comparisons. The meta-analysis included 11 articles for a total of 575 children: the pooled prevalence of AIHA was greater in (1) IgG aCL-positive than IgG aCL-negative children (39.7% vs 20.9%, p = 0.005); (2) in APS-positive than APS-negative SLE children (36.8% vs 13.2%, p = 0.01); and (3) in SLE-related APS than in primary APS children (53% vs 16.2%, p = 0.008). The pooled prevalence of AIHA is greatest in SLE with aPL/APS, low-moderate in SLE without aPL/APS, and lowest in primary APS. Key Points • Antiphospholipid antibodies strongly relate to autoimmune haemolytic anaemia. • Autoimmune haemolytic anaemia is more common in systemic lupus erythematosus with antiphospholipid antibodies.

The Analysis of Clinical Features of Systemic Lupus Erythematosus (SLE) in Children

Objective: Observe the clinical characteristics of children with SLE, namely, to observe the symptoms and laboratory examinations, such as blood routine, blood lipid, immunoglobulin, complement, autoantibodies, serum 25 (OH) D and other indicators, and to explore the clinical characteristics, the difference and the significance of vitamin D supplements between male and female SLE patients in children respectively. Methods: We enrolled 64 cases of SLE patients in children who were admitted into the department of pediatrics and rheumatology of the third affiliated hospital of sun yat-sen university in guangzhou from May 1, 2011 to February 1, 2019, They were analyzed retrospectively, adopting ?² test for statistical analysis. Results: 64 cases of SLE in children, which included 10 cases of male and 54 cases of female. Clinical manifestations: facial skin rash in 48 patients (75%), fever in 38 cases (59.4%), arthritis in 28 cases (43.8%), oral ulcer in 18 cases (28.1%), serositis in13 cases (20.3%), and the sun allergy in 9 cases (14.1%), the damage of central nervous system in 7 cases (10.9%) . Laboratory examination: 30 cases of leukopenia (46.9%), anemia in 30 cases (46.9%), thrombocytopenia in 12 cases (18.8%), hematuria in 18 cases (28.1%), proteinuria in 33 cases (51.2%), 6 patients with renal impairment (9.4%), antinuclear antibody positive in 63 cases (98.4%), anti-double-stranded DNA (dsDNA) antibody positive in 48 cases (75%), anti SSA antibody positive in 44 cases (68.7%), SSB antibody positive in 33 cases (51.6%), Sm antibody positive in 40 cases (62.5%), nucleosome antibody positive in 28 cases (43.8%) .Among these children, male SLE patients were higher than female children with SLE in the damage of kidney, Sm antibodies and resisting nucleosome antibody positive rates (?²= 4.451, 8.336, 6.803, P&lt;0.05), the female children with SLE was higher than male SLE Children in the anti-SSB antibody positive rate (?²=4.945, P &lt; 0.05). In 64 cases of SLE children, which included 52 cases were lower than the normal level of serum 25 (OH) D measurements, 12 cases were in the normal lower limit of serum 25 (OH) D measurements, at the same time, the female SLE. Patients was higher than male children with SLE in the reduce rate of serum 25 (OH) D (?²= 8.351, P &lt; 0.05). Conclusion: Male SLE patients which appeared damage of kidney easier than female patients , the proteinuria was the most common in the damage of kidney. Resistance to Sm antibodies which was the risk factor of renal injury with higher incidence in male children with SLE; Anti nucleosome antibody which was the risk factor for the disease activity in male children with SLE were higher than female children with SLE. It was estimated that the risk of Sjogren's syndrome appeared in female with SLE were higher than that in male SLE children. In this retrospective study, the serum 25 (OH) D levels were significantly lower in children with SLE, and vitamin D supplementation was required.

CD40 and CD72 expression and prognostic values among children with systemic lupus erythematosus: a case-control study.

Systemic lupus erythematosus (SLE) is a chronic disease with proven interactions between immune system components, including both humoral- and cell-mediated immunity, as well as co-stimulatory and inhibitory molecules such as CD40 and CD72. Here, we investigated CD40 and CD72 expression on B cells of SLE children and assessed their prognostic values. We conducted a preliminary case-control study in Mansoura University Children's Hospital, Egypt from September 2018 to January 2020 including 27 SLE children and 27 healthy controls. We assessed cases during initial flare and after remission. Flow cytometry analysis was carried out for all participants for CD40 and CD72 expression of B cells. During flare, SLE cases had statistically significant higher CD40 and lower CD72 expression in comparison with controls (p < 0.001). After remission, the number of CD40+ B cells significantly decreased (p < 0.001), while the number of CD72+ B cells significantly increased (p < 0.001) in comparison with flare. We reported non-significant positive correlations between CD40 expression and SLE Disease Activity Index (SLEDAI; p = 0.347 during flare and p = 0.653 after remission) and negative correlations between CD72 expression and SLEDAI (p = 0.34 during flare and p = 0.044 after remission). No significant differences were detected between renal histopathology classes with regard to CDs expression on B cells (p = 0.45 for CD40 and p = 0.63 for CD72). In conclusion, CD40+ B cells and CD72+ B cells could be considered as markers of paediatric SLE flare and remission, respectively.

Serum RANKL, osteoprotegerin (OPG) and RANKL/OPG ratio in children with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) patients have lower bone mineral density (BMD) compared with healthy individuals because of general, genetic, disease and medication-related factors. The disturbance of the receptor activator of nuclear factor-κB ligand (RANKL)/osteoprotegerin (OPG) ratio has been reported to be associated with low BMD in many disorders in adults and children alike. The objectives of this study were (i) to assess serum OPG, RANKL and RANKL/OPG ratio levels in SLE children and controls, (ii) to determine whether the cumulative glucocorticoid (CGCS) dose had any effect on the concentration of serum RANKL, OPG and RANKL/OPG ratio, and (iii) to determine the relation of these parameters to BMD. We evaluated 50 SLE children and 50 age- and sex-matched healthy controls. RANKL and OPG were assessed in serum and compared between patients and controls. For SLE patients, a univariate followed by multivariable analysis were carried out to detect the possible predictors of the changes in RANKL, OPG and RANKL/OPG ratio levels. Lumbar BMD for all patients was assessed by dual-energy X-ray absorptiometry (DXA) scan and then correlated to different probable correlated factors. RANKL, OPG and RANKL/OPG ratio were significantly higher in SLE patients (p ≤ 0.001). Univariate analysis showed significant correlations of RANKL with CGCS (p ≤ 0.001) and with DXA scan z-score (p = 0.007): OPG was significantly correlated to Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (p = 0.001) and anti-double-stranded DNA (p = 0.001), whereas RANKL/OPG was significantly correlated to duration of illness and DXA z-score (p = 0.002). The multivariable analysis showed that DXA z-score was an independent predictor of RANKL and RANKL/OPG ratio (p = 0.019 and 0.008, respectively), whereas SLEDAI score was an independent predictor of OPG levels. BMD was negatively correlated to disease duration (p = 0.008) and CGCS dose (p = 0.015), but no significant correlation has been found between BMD and cumulative SLEDAI score (p = 0.29). Serum RANKL/OPG ratio is elevated in Egyptian children with SLE and is considered a risk factor for reduced bone mass in these children. Other risk factors for low BMD include high CGCS dose and disease duration, supporting that osteoporosis in SLE is multifactorial.

Oxidative stress and vitamin D receptor BsmI gene polymorphism in Egyptian children with systemic lupus erythematosus: a single center study.

Systemic lupus erythematosus (SLE) is an autoimmune disease with unknown exact etiology. Vitamin D receptor gene ( VDR) and oxidative stress play important roles in the pathogenesis of SLE. Here we investigated the genotypes and allelic frequencies of VDR BsmI polymorphism as well as their relationship with oxidative stress markers in Egyptian SLE children. We conducted a cross-sectional comparative study at Mansoura University Children's Hospital, Egypt from 2014 to 2018 including 100 SLE children and 100 controls. We investigated both groups for VDR BsmI polymorphism using polymerase chain reaction. Oxidative stress was assessed using malondialdehyde, glutathione S-transferase, superoxide dismutase, catalase and total antioxidant capacity. BB genotype frequency was found to be significantly higher in the SLE group ( p = 0.04, odds ratio (95% confidence interval) = 2.5 (1.01-5.9)). However, VDR B allele and b allele showed insignificant differences between SLE patients and controls ( p = 0.36, odds ratio (95% confidence interval) = 1.2 (0.8-1.8)). Lower levels of glutathione S-transferase, superoxide dismutase and total antioxidant capacity were found in the SLE group with statistically significant differences as regards glutathione S-transferase and superoxide dismutase ( p < 0.001). Serum malondialdehyde and catalase levels were significantly higher in the SLE group ( p < 0.001). No significant differences were found between VDR BsmI polymorphism (genotypes and alleles) and oxidative stress markers in the SLE group. In conclusion, BB genotype of VDR BsmI polymorphism is associated with an increased risk of SLE among Egyptian children. Oxidative stress may contribute in pathogenesis of SLE but is not associated with VDR BsmI polymorphism.

Natural Killer and Natural Killer T Cells in Juvenile Systemic Lupus Erythematosus: Relation to Disease Activity and Progression.

The contribution of innate immune cells, including natural killer (NK) and natural killer T (NKT) cells, in systemic lupus erythematosus (SLE) is still unclear. Herein, we examined the frequency of peripheral NK cells, CD56dim and CD56bright NK cells, and NKT cells in patients with juvenile SLE and their potential relations to SLE-related clinical and laboratory parameters. The study included 35 SLE children and 20 apparently healthy controls. After baseline clinical and lab work, SLE Disease Activity Index (SLEDAI-2K) and Pediatric Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (Ped-SDI) scores were assessed. The frequency of peripheral NK cells, CD56dim and CD56bright NK cells, and NKT cells was examined using flow cytometry. SLE patients showed significantly lower frequency of NK cells and NKT cells and higher frequency of CD56bright NK cells compared to controls. Disease activity, urea, and creatinine correlated negatively with NK, but positively with CD56bright NK cells. NK and NKT cells exhibited inverse correlation with the renal biopsy activity index; however, CD56bright NK cells showed direct correlations with both activity and chronicity indices. Regarding Ped-SDI, renal, neuropsychiatry disorders, and growth failure correlated inversely with NK but directly with CD56bright NK cells. NKT cell inversely correlated with renal damage and delayed puberty. In conclusion, low frequency of NK and NKT and expansion of CD56bright NK cells are marked in juvenile SLE, particularly with activity. These changes have direct effect on renal impairment and growth failure, reflecting their potential influence on disease progression.

Evaluation of Dry Eye Disease in Children With Systemic Lupus Erythematosus and Healthy Controls.

To compare the symptoms and signs of dry eye disease (DED) in children with systemic lupus erythematosus (SLE) with those in healthy children using common diagnostic tools. Prospective, observational, single-center cohort study. Thirty-four subjects with SLE and 15 healthy subjects were recruited from the Hospital for Sick Children in Toronto, Canada. Subjects underwent subjective and objective dry eye assessments using the Canadian Dry Eye Assessment (CDEA) questionnaire, tear film osmolarity, slit lamp examination, tear film break-up time, corneal fluorescein staining, Schirmer test 1, and conjunctival lissamine green staining. No difference in symptoms was found between children with SLE and healthy children (CDEA score 6.4 ± 5.4 vs. 3.8 ± 3.2; P = 0.09). Corneal staining was more prevalent in children with SLE than in healthy children (58.8% vs. 20.0%; P = 0.01), and children with SLE had higher mean corneal fluorescein staining scores (1.7 ± 1.7 vs. 0.2 ± 0.4; P = 0.002). No statistically significant differences in tear osmolarity, inter-eye differences in tear osmolarity, tear film break-up time, Schirmer test 1, or lissamine green staining scores were observed between the 2 groups. In healthy children, CDEA scores weakly correlated with corneal fluorescein staining score (r = 0.53, P = 0.04). In children with SLE, no correlation between CDEA score and any of the diagnostic test outcomes was found. There is discordance between symptoms and signs of DED in children with SLE. Corneal fluorescein staining is essential for the diagnosis of DED in these children.

Microarray expression profile of circular RNAs and mRNAs in children with systemic lupus erythematosus.

Recently, it was reported that circular RNAs (circRNAs) play the crucial role in many physiological and biological processes and can be used as biomarkers. However, the information about circRNAs in children with systemic lupus erythematosus (SLE) is limited. The aim of this study is to determine the expression of circRNAs in children with SLE and investigate the significance of circRNA for diagnosing SLE. Microarray profile of circRNAs and mRNAs was performed for identifying the changes in expression of circRNAs and mRNAs between children with SLE and healthy children. Quantitative polymerase chain reaction (qPCR) was used to confirm the results. Spearman correlation test was performed to assess the correlation between circRNAs and clinical variables. The receiver operating characteristic (ROC) curve was calculated for evaluating the diagnostic value. A comparison between the children with SLE and healthy children revealed that 348 circRNAs and 1162 mRNAs were expressed differentially. The authors constructed a complex circRNA target network consisting of 307 matched circRNA-mRNA pairs for 124 differentially expressed circRNAs (74 circRNAs were upregulated, and 50 circRNAs were downregulated) and 142 differentially expressed mRNAs (83 mRNAs were upregulated, and 59 mRNAs were downregulated) by using gene co-expression network analysis. The competing for endogenous RNA (ceRNA) network includes 42 differentially expressed circRNAs, 41 differentially expressed mRNAs, and 71 predicted miRNAs. Among these SLE patients, we detected that the hsa_circ_0021372 and hsa_circ_0075699 levels are associated with C3 and C4 levels in children with SLE. The hsa_circ_0057762 level is positively associated with the SLEDAI-2K score. The ROC curves of circRNAs showed that the levels of hsa_circ_0057762 (AUC 0.804, 95% CI 0.607-1.0, P = 0.02) and hsa_circ_0003090 (AUC 0.848, 95% CI 0.688-1.0, P = 0.008) could differentiate the patients with SLE from the healthy controls. We firstly characterized the expression profiles of circRNA and mRNA in children with SLE and propose herein their possible roles in the pathogenesis of SLE. These results provide novel insight into the mechanisms of SLE pathogenesis, and circRNAs may serve as useful biomarkers for SLE.

Medical compliance behavior among systemic lupus erythematosus children at a ClassIII Grade A hospital in Beijing

Objective To explore the medical compliance behavior among systemic lupus erythematosus (SLE) children so as to provide a basis for implementing targeted nursing intervention. Methods From November 2015 to October 2017, we selected 124 SLE children of Department of Pediatrics of Peking Union Medical College Hospital as subjects in by convenience sampling. The self-designed SLE Children Medical Compliance Behavior Questionnaire was used to the investigation. A total of 124 questionnaires were sent out and 116 valid questionnaires were collected. Results Among 116 children, the mean of total score of medical compliance behavior was (105.91±10.62) along with the highest score (4.91±0.43) in the term of I will not presume to reduce the dose belong to the dimension of medication and review. Conclusions SLE children have the high level of compliance in medication and review. Health care providers should continue to carry out sunscreen, choose appropriate diet, pay attention to the details of infection prevention and develop a regular exercise habit for children along with their patients so as to improve long-term prognosis and quality of life of SLE children. Key words: Child; Lupus erythematosus, systemic; Patient compliance

Clinically diagnosed urticaria and risk of systemic lupus erythematosus in children: A nationwide population-based case-control study.

Urticaria is one of the most common diseases seen in clinical practice, whereas several reports have proposed that urticaria may have a link with autoimmune disorders. Few studies have examined the clinical association between urticaria with systemic lupus erythematosus (SLE). By conducting a nationwide population-based case-control study in Taiwan, we evaluated the risk of SLE in children with a prior clinical diagnosis of urticaria. Using 2000-2011 claims data from the Taiwanese National Health Insurance Research Database, we identified 2105 SLE children during 2004-2011 as the study group, along with randomly selected 8420 non-SLE patients matched (1:4) for age, sex, and first diagnosis date as the control group. The correlation between urticaria and SLE risk was estimated using conditional logistic regression analysis. The prevalence rates of clinically diagnosed acute and chronic urticaria in SLE patients were 22.09% and 18.24%, respectively. A significant association was found between clinically diagnosed urticaria and childhood SLE, with a stronger risk associated with more episodes of urticaria (≥3 visits, OR: 2.33, 95% CI 1.91-2.84). The risk was higher with chronic urticaria (OR: 2.21, 95% CI 1.85-2.64) than with acute urticaria (OR: 1.54, 95% CI 1.34-1.76). Subgroup analysis stratified by sex or age indicated that the risk associated with SLE was significantly greater among female children and adolescents with urticaria. Our results suggest that children with urticaria have a significantly higher risk of SLE, with the risk increasing further among those with more episodes of urticaria or chronic urticaria.

Exhaled nitric oxide, pulmonary function, and disease activity in children with systemic lupus erythematosus.

To determine the association among fractional exhaled nitric oxide (FENO), pulmonary function, and disease activity in children with systemic lupus erythematosus (SLE). Children aged 7-18 years, diagnosed with SLE under the criteria of the American Rheumatism Association (revised 2012), were enrolled. All eligible participants had disease activity, FENO, and pulmonary function evaluated and re-evaluated at 6-month follow-up. Twenty-four children (95.8% female; mean age 15.2 ± 2 years; median disease duration 2.4 years) were studied. The mean FENO1 and FENO2 were 19.6 ± 7.2 parts per billion (ppb) and 17.4 ± 4.5 ppb, respectively. At baseline, 20.8% had abnormal pulmonary functions (all restrictive defects) and increased to 29.2% at follow-up (isolated restrictive defect 25% and restrictive with diffusion defect 4.2%). Most of their disease activities at baseline and second assessment were non-active (58.3%, 70.8%) or mild disease activities (20.8% both). There was significant correlation between FENO and disease activity (r = 0.49; P-value = 0.02). The significant negative correlation between total lung capacity (TLC) and disease activity was detected in children with active SLE (r = -0.71; P-value = 0.02). Decreased TLC and high FENO were common in SLE children who had no respiratory symptoms and correlated with disease activity. FENO should be considered as an additional pulmonary function to evaluate disease activity in children with SLE.

Juvenile lupus: Different clinical and serological presentations compared to adult lupus in Egypt

Aim of the work: We aimed to evaluate the differences in clinical presentation, serological pattern and disease activity between juvenile and adult-onset of Egyptian systemic lupus erythematosus (SLE) patients. Patients and methods: 160 Egyptian SLE patients (80 Adult-onset and 80 juveniles) were included. Patients records were reviewed for clinical and laboratory evaluation on presentation. Disease activity at onset was assessed using SLE Disease Activity Index (SLEDAI). Results: The mean age of the adult patients was 29.9±7.2years and of the juvenile cases (12.8±2.1years). The female:male ratio of the adults was 10:1 while it was 39:1 in the SLE children. The most common clinical presentation among adult SLE was malar rash (75%) followed by articular manifestations (62.5%), while in juveniles, nephritis (78.8%) followed by articular manifestations (71.2%) were the most common. Juvenile patients had more frequent neuropsychiatric (p=0.015) and hematologic abnormalities (p<0.001) at onset; and lupus nephritis (72.5%) compared to adults (36.2%) (p<0.001) during the first year of presentation. Juvenile SLE showed higher frequency of proteinuria (p<0.001), hematuria (p=0.02) and active urinary sediments (p=0.016). Proliferative nephritis was the most common form among both juveniles and adults. Positivity and titres of both anticardiolipin antibodies and lupus anticoagulant were significantly higher in juvenile SLE. Juvenile SLE patients had significantly higher SLEDAI [median (IQR): 12 (10–22)] compared to adults [median (IQR): 8 (4–12)], p<0.001. Conclusion: Juvenile SLE patients differ from adult SLE with more frequent major organs affection and significantly higher serological activity. Earlier and more careful assessment with strict management plan and follow-up are needed in juvenile SLE patients.

Immunogenicity of 23-Valent Pneumococcal Vaccine in Children with Systemic Lupus Erythematosus.

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease which is characterized by B-cell abnormality and auto-antibody generation. Since bacterial infections are the most important causes of mortality in these patients, pneumococcal vaccination is recommended for children with SLE. To investigate humoral immunity and specific-antibody formation in response to a 23-valent polysaccharide pneumococcal vaccination in SLE children and asthmatic control group. The case and control groups consisted of 30 children with the mean age of 13 years who were matched by sex and age. Anti-pneumococcal antibody titers were determined using Enzyme-Linked Immunosorbent Assay (ELISA) before the vaccination with the 23-valent pneumococcal vaccine and 3 weeks later in both groups. Also the correlation between anti-pneumococcal antibody titer and different factors including age, sex, lupus activity, disease duration, medications, history of recurrent infections, and laboratory data were investigated. Both groups showed significant increases in anti-pneumococcal antibody level after vaccination (p≤0.001). The increase in antibody level were almost the same in both groups (p≥0.05) such that 77.7% of SLE children and 86.2% of control children showed at least 2-fold increase in anti-pneumococcal antibody titer following immunization. Significant correlations were seen between the level of post-immunization anti-pneumococcal antibody with the age of children with SLE (p=0.02) and their age of disease onset (p=0.02). It is concluded that pneumococcal vaccination is generally immunogenic in children with SLE. However, a small group of patients show impaired response to the vaccine.

The unusually high occurrence of hematological abnormalities in pediatric-SLE in Saudi children - possible causes and diagnostic significance

Systemic Lupus Erythematosus (SLE) is an autoimmune disease with a highly variable and complex symptomatology. Indeed many clinicians working with SLE regard it as a group of diseases [1]. In SLE, autoantibodies are formed against DNA, protein and phospholipid antigens leading to the generation of inflammation and damage in many types of tissue including skin, joints, kidneys, lungs and the nervous system. Depending on the clinical presentation, an SLE patient may be referred to a dermatologist, rheumatologist, hematologist, nephrologist, neurologist or psychiatrist [1], receiving different treatment from each specialist. To complicate matters further, SLE is characterized by periods of well-being interspersed with disease flares. The prognosis had improved greatly in the last 60 years. In the early 1950s, the 5 years survival rate for SLE was 50%, but now it is higher than 90% [2]. This dramatic improvement is due to physicians’ diagnostic skills and to the early administration of immunosuppressive drugs, typically corticosteroids, hydroxychloroquine, methotrexate or more recently, Rituximab, a chimeric monoclonal antibody directed against CD20 on the B-cell surface. Prompt diagnosis is crucial to patient prognosis, especially maintenance of renal function [3]. However, SLE presents with such a variety of symptoms that diagnosis is not at all straightforward. In this issue of The Journal of Pediatric Biochemistry, Mohammed Muzaffer reports hematological data from a study of Saudi Arabian children with SLE (pediatric SLE). A remarkable finding of this study is that hematological abnormalities were present in 83% (30 of 36 children) of pediatric SLE (p-SLE) children. As Muzaffer points out, this occurrence is much higher than other studies have found in p-SLE in the USA, France and the Middle East, including Saudi Arabia and Kuwait. A recent study in Kuwait [4] found that only 20% of children were anemic, whereas Muzaffer’s study in Jeddah, Saudi Arabia, finds anemia in 80% of his group. Clearly this large difference in the clinical presentation of p-SLE in two geographically juxtaposed and ethnically related countries may indicate something important about pediatric SLE etiology. Genome wide association studies have confirmed more than 20 robust associations of loci in the human genome with susceptibility to SLE [5] and estimates put the total number of SLE-associated genes at as much as 100. Curiously, the genetic architecture of SLE has more in common with Crohn’s disease, which also has