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Background: Systemic Lupus Erythematosus (SLE) patients are more likely than the general population to suffer from thyroid illness. The major goal was to assess the thyroid dysfunctions due to immunological factors in Egyptian SLE children and how they are related to the course and severity of the illness. Methods: Fifty children and adolescents with SLE are included in this cross-sectional observational study. Every patient underwent a thorough physical examination and a comprehensive history taking. An enzyme-linked immunosorbent assay (ELISA) approach was used to evaluate the thyroid profile, anti-thyroglobulin (Anti-TG), and anti-thyroid peroxidase (anti-TPO) antibodies. Results: Of the 50 patients, the female: male ratio (F: M = 7:1) was 44 females and 6 males (12%). They were between the ages of 5 and 17. Out of the patients, thirty-two (64%) had thyroid dysfunctions, 19 (38%) had euthyroid sick syndrome, ten (20%) had overt hypothyroidism, three (6%) had subclinical hypothyroidism, and none had hyperthyroidism. Of the 50 patients, one (2%) had increased anti-TPO, whereas all other patients had normal anti-TG levels. A statistically significant negative correlation (p-value 0.007) was seen between the disease duration and free thyroxine (FT4). Furthermore, a significant negative correlation (p-values 0.015 and 0.028) was found when comparing the disease duration with thyroid antibodies (anti-TG and anti-TPO). Conclusion: In Juvenile Systemic Lupus Erythematosus (JSLE), thyroid dysfunctions can be identified. The disease duration but not its activity was significantly correlated with thyroid antibodies. For children with JSLE, thyroid function testing should be done on a regular basis. It is preferable to carry out additional thyroid antibody tests when necessary. [ABSTRACT FROM AUTHOR]

Introduction: We aimed to analyze the thicknesses of the retinal sublayer and peripapillary retinal nerve fiber layer (pRNFL) in patients with juvenile systemic lupus erythematosus (JSLE) without lupus retinopathy. Methods: Thirty-six patients with JSLE (36 eyes) and 30 control subjects (30 eyes) were included retrospectively. Demographic data, disease duration, and clinical manifestations were recorded. Optical coherence tomography was used to examine the macula and optic disc. The thicknesses of the retina, ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer (ONL), retinal pigment epithelium (RPE), and pRNFL were measured. The correlation between the thickness of retina and disease duration, erythrocyte sedimentation rate (ESR) were investigated. Results: The retinal thicknesses of I3 and T3 were thinner in the JSLE group than in the control group (P = 0.019, P = 0.043, respectively). The thicknesses of the I3 and S6 sectors of the GCL decreased significantly (P = 0.013, and P = 0.022, respectively). The thickness of the S6 sector of the IPL was reduced in the JSLE group compared with the control group (P = 0.047). The JSLE group showed significant decrease in the thickness of the central sector of the ONL (P = 0.034). No statistically significant differences in INL, OPL, RPE, and pRNFL thicknesses were found. The retinal thicknesses of I3 (r = -0.386, P = 0.020) and T3 (r = -0.384, P = 0.021) presented negative associations with ESR, but had no significant correlations with disease duration. Conclusions: Retinal thinning was observed in patients with JSLE without lupus retinopathy, and this change was more pronounced in the inner layer. Key Points • Retinal thinning occurs in JSLE patients without lupus retinopathy. • Changes in retinal thicknesses are related to the ESR. [ABSTRACT FROM AUTHOR]

Pyogenic granuloma (PG) is a benign vascular neoformation, presenting as a painful red nodule on the skin, mucosa or nail apparatus. It is usually related to local complications such as bleedings and superinfections. The etiology of PG remains still unclear, and several triggers can lead to its formation. In case of multiple lesions, systemic conditions and drugs remain the main causes. Antineoplastic treatments, retinoids, antiretrovirals, hormones and anticonvulsants are frequently implicated in PG formation. In literature, PG has been rarely described in the course of biological treatment due to rheumatological disease. The present case report describes the development of polydactolous PGs in a 21-year-old woman with juvenile systemic lupus erythematosus (jSLE) during treatment with belimumab, a monoclonal antibody directed against BlyS. The clinical presentation, in particular the timing and the multiplicity of the lesions, and the improvement after belimumab discontinuation allowed us to consider PG as drug-induced. This case highlights the importance of considering PG as a potential complication of rheumatologic treatments. [ABSTRACT FROM AUTHOR]

Abstract Background Systemic Lupus Erythematosus (SLE) patients are more likely than the general population to suffer from thyroid illness. The major goal was to assess the thyroid dysfunctions due to immunological factors in Egyptian SLE children and how they are related to the course and severity of the illness. Methods Fifty children and adolescents with SLE are included in this cross-sectional observational study. Every patient underwent a thorough physical examination and a comprehensive history taking. An enzyme-linked immunosorbent assay (ELISA) approach was used to evaluate the thyroid profile, anti-thyroglobulin (Anti-TG), and anti-thyroid peroxidase (anti-TPO) antibodies. Results Of the 50 patients, the female: male ratio (F: M = 7:1) was 44 females and 6 males (12%). They were between the ages of 5 and 17. Out of the patients, thirty-two (64%) had thyroid dysfunctions, 19 (38%) had euthyroid sick syndrome, ten (20%) had overt hypothyroidism, three (6%) had subclinical hypothyroidism, and none had hyperthyroidism. Of the 50 patients, one (2%) had increased anti-TPO, whereas all other patients had normal anti-TG levels. A statistically significant negative correlation (p-value 0.007) was seen between the disease duration and free thyroxine (FT4). Furthermore, a significant negative correlation (p-values 0.015 and 0.028) was found when comparing the disease duration with thyroid antibodies (anti-TG and anti-TPO). Conclusion In Juvenile Systemic Lupus Erythematosus (JSLE), thyroid dysfunctions can be identified. The disease duration but not its activity was significantly correlated with thyroid antibodies. For children with JSLE, thyroid function testing should be done on a regular basis. It is preferable to carry out additional thyroid antibody tests when necessary.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems, including the central nervous system. Depression is one of the neuropsychiatric manifestations of juvenile SLE . Objective To estimate the prevalence of depressive disorders in juvenile SLE and identify its potential risk factors. Methods This cross-sectional study was conducted in juvenile SLE patients at Dr. Mohammad Hoesin General Hospital, Palembang. Sociodemographic data and medications were recorded. Disease activity of SLE was assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Physical activity was measured using the Physical Activity Questionnaire for Children (PAQ-C) and the Physical Activity Questionnaire for Adolescents (PAQ-A). All subjects were screened for depression using the Childhood Depression Inventory (CDI) questionnaire. Multiple linear regression analyses were used to determine risk factors for depressive disorders. Results We included 72 patients, of whom 67 (93.1%) were female. Mean age of the patients was 12 years 4 months (SD 2 years 6 months); most (67; 93.1%) were 10-18 years of age. Depression was found in 24 patients (33.3%). SLEDAI scores of ?3 (flare) were found in 54 patients (75%) and low physical activity was found in 62 (86.1%) patients. The prevalence of depression based on the Children's Depression Inventory was 33.3%. A SLEDAI score of ?3 and low physical activity were significantly associated with depression (P=0.009 and P=0.025, respectively). On multiple linear regression analysis, only SLEDAI score of ?3 remained significantly associated with depression (P= 0.017; OR 12.6; 95%CI 1.6–101.7). Gender, age, family economic status, father's education, mother's education, family history of depression, and duration of illness were not associated with depression. Conclusion A SLEDAI score indicating flare (?3) and low physical activity are significantly associated with an increased risk of depression. A SLEDAI score is an independent risk factor for depression when all other significant risk factors are considered.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems, including the central nervous system. Depression is one of the neuropsychiatric manifestations of juvenile SLE. Objective To estimate the prevalence of depressive disorders in juvenile SLE and identify its potential risk factors. Methods This cross-sectional study was conducted in juvenile SLE patients at Dr. Mohammad Hoesin General Hospital, Palembang. Sociodemographic data and medications were recorded. Disease activity of SLE was assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Physical activity was measured using the Physical Activity Questionnaire for Children (PAQ-C) and the Physical Activity Questionnaire for Adolescents (PAQ-A). All subjects were screened for depression using the Children's Depression Inventory (CDI) questionnaire. Multiple linear regression analyses were used to determine risk factors for depressive disorders. Results We included 72 patients, of whom 67 (93.1%) were female. Mean age of the patients was 12 years 4 months (SD 2 years 6 months); most (67; 93.1%) were 10-18 years of age. Depression was found in 24 patients (33.3%). The SLEDAI scores of =3 (flare) were found in 54 patients (75%) and low physical activity was found in 62 (86.1%) patients. The prevalence of depression based on the CDI was 33.3%. A SLEDAI score of =3 and low physical activity were significantly associated with depression (P=0.009 and P=0.025, respectively). On multiple linear regression analysis, only SLEDAI score of =3 remained significantly associated with depression (P= 0.017; OR 12.6; 95%CI 1.6 to 101.7). Gender, age, family economic status, father's education, mother's education, family history of depression, and duration of illness were not associated with depression. Conclusion A SLEDAI score indicating flare (=3) and low physical activity are significantly associated with an increased risk of depression. A SLEDAI score is an independent risk factor for depression when all other significant risk factors are considered. [ABSTRACT FROM AUTHOR]

Abstract Background In juvenile systemic lupus erythematosus (j-SLE) with neuropsychiatric (NP) symptoms, there is a lack of diagnostic biomarkers. Thus, we study whether PET-FDG may identify any metabolic dysfunction in j-NPSLE. Methods A total of 19 18FDG-PET exams were consecutively performed using PET-MRI system in 11 non-sedated patients presenting with j-NPSLE (11-18y) for less than 18 months (m) and without any significant lesion at MRI. Psychiatric symptoms were scored from 0 (none) to 3 (severe) at PET time. PET images were visually analyzed and voxel-based analyses of cerebral glucose metabolism were performed using statistical parametric mapping (spm) with an age-matched control group, at threshold set > 50 voxels using both p

Objective: Juvenile systemic lupus erythematosus (j-SLE) is a rare chronic auto-immune disease involving several organs. Neuropsychiatric (NP) SLE (NPSLE) is frequent in j-SLE and associated with increased morbidity/mortality. Although NPSLE classification criteria exist, attributing NP features to j-SLE remains a major challenge. The study objective is to thoroughly describe j-NPSLE patients and assist in their diagnosis. Methods: This is a 4-year retrospective monocentric study of j-SLE patients. NP events were attributed to j-SLE using standardised diagnostic criteria and multidisciplinary paediatric clinical expertise. Clinical features, brain magnetic resonance imaging (MRI)s and samples analysis including cerebrospinal fluid were assessed. A risk of j-NPSLE score was developed based on multivariable logistic regression analysis. Results: Of 39 patients included, 44% were identified as having j-NPSLE. J-NPSLE diagnosis was established at the onset of j-SLE in 59% of patients. In addition to frequent kidney involvement (76%) and chilblains (65%), all j-NPSLE patients displayed psychiatric features: cognitive symptoms (82%), hallucinations (76%), depressed mood (35%), acute confused state (18%) and catatonia (12%). Neurological involvement was often mild and nonspecific, with headache (53%) in about half of the patients. The main features reported on brain MRI were nonspecific T2/FLAIR white matter hyperintensities (65%), and cerebral atrophy (88%). Upon immunosuppressive treatment, clinical improvement of NP features was observed in all j-NPSLE patients. The score developed to attribute j-NPSLE probability, guide further investigations and appropriate treatments is based on hallucinations, memory, sleep and renal involvement (Sensitivity: 0.95 Specificity: 0.85). Cerebrospinal fluid (CSF) neopterin assessment increases the score sensitivity and specificity. Conclusion: Physicians should carefully and systematically assess the presence of NP features at diagnosis and early stages of j-SLE. For j-NPSLE patients with predominant psychiatric features, a multidisciplinary collaboration, including psychiatrists, is essential for the diagnosis, management and follow-up. [ABSTRACT FROM AUTHOR]

Background: In juvenile systemic lupus erythematosus (j-SLE) with neuropsychiatric (NP) symptoms, there is a lack of diagnostic biomarkers. Thus, we study whether PET-FDG may identify any metabolic dysfunction in j-NPSLE. Methods: A total of 19 18FDG-PET exams were consecutively performed using PET-MRI system in 11 non-sedated patients presenting with j-NPSLE (11-18y) for less than 18 months (m) and without any significant lesion at MRI. Psychiatric symptoms were scored from 0 (none) to 3 (severe) at PET time. PET images were visually analyzed and voxel-based analyses of cerebral glucose metabolism were performed using statistical parametric mapping (spm) with an age-matched control group, at threshold set > 50 voxels using both p < 0.001 uncorrected (unc.) and p < 0.05 corrected family wise error (FWE). Results: Patients exhibited mainly psychiatric symptoms, with diffuse inflammatory j-NPSLE. First PET (n = 11) was performed at a mean of 15y of age, second/third PET (n = 7/n = 1) 6 to 19 m later. PET individual analysis detected focal bilateral anomalies in 13/19 exams visually but 19/19 using spm (unc.), mostly hypermetabolic areas (18/19). A total of 15% of hypermetabolic areas identified by spm had been missed visually. PET group analysis (n = 19) did not identify any hypometabolic area, but a large bilateral cortico-subcortical hypermetabolic pattern including, by statistical decreasing order (unc.), thalamus, subthalamic brainstem, cerebellum (vermis and cortex), basal ganglia, visual, temporal and frontal cortices. Mostly the subcortical hypermetabolism survived to FWE analysis, being most intense and extensive (51% of total volume) in thalamus and subthalamus brainstem. Hypermetabolism was strictly subcortical in the most severe NP subgroup (n = 8, scores 2–3) whereas it also extended to cerebral cortex, mostly visual, in the less severe subgroup (n = 11, scores 0–1), but difference was not significant. Longitudinal visual analysis was inconclusive due to clinical heterogeneity. Conclusions: j-NPSLE patients showed a robust bilateral cortico-subcortical hypermetabolic network, focused subcortically, particularly in thalamus, proportionally to psychiatric features severity. Further studies with larger, but homogeneous, cohorts are needed to determine the sensitivity and specificity of this dysfunctional pattern as a potential biomarker in diffuse inflammatory j-NPSLE with normal brain MRI. [ABSTRACT FROM AUTHOR]

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystem inflammatory disease that manifests before the age of 16 years, following a remitting - relapsing course. The clinical presentation in children is multifaceted, most commonly including constitutional, hematological, cutaneous, renal, and neuropsychiatric symptoms. Neuropsychiatric manifestations range widely, affecting approximately 14–95 % of jSLE patients. They are associated with high morbidity and mortality, particularly at a younger age. Headaches, seizures, cognitive dysfunction, and mood disorders are the most frequent neuropsychiatric manifestations. The pathophysiological mechanism is quite complex and has not yet been fully investigated, with autoantibodies being the focus of research. The diagnosis of neuropsychiatric jSLE remains challenging and exclusionary. In this article we review the clinical neuropsychiatric manifestations associated with jSLE with the aim that early diagnosis and prompt treatment is achieved in children and adolescents with the disease. [ABSTRACT FROM AUTHOR]

Objective: The aim is to evaluate subclinical alterations of macular microvasculature in patients with juvenile systemic lupus erythematosus (JSLE). Methods: This is a cross-sectional study of 29 eyes of 29 patients diagnosed with JSLE and 29 eyes of 29 healthy controls. The vessel density (VD) of the superficial capillary plexus (SCP), intermediate capillary plexus (ICP), deep capillary plexus (DCP), choriocapillaris (CC), and area of foveal avascular zone (FAZ) was measured using optical coherence tomography angiography (OCTA). A multiple linear regression analysis was performed to evaluate the effects of disease duration and activity on OCTA parameters. Results: The VD of total (p =.007) and the superior (p =.014) and inferior (p =.004) quadrants in SCP was significantly lower in children with JSLE. The VD of total and all quadrants in ICP decreased (p =.015, p =.0045, p =.015, p =.033), except that of the temporal quadrant (p =.366). The total (p =.011) and superior quadrant (p <.01) DCP-VD showed a significant decrease in children with JSLE. The decrease in VD in the total (p =.003) and nasal quadrant (p =.017) of CC was also remarkable. No significant difference in the FAZ area was found between the two groups (p =.774). Multiple linear regression analyses adjusted for age, spherical equivalent, and intraocular pressure were conducted. No contributing factor to OCTA parameters was found. Conclusions: We demonstrated decreased VD in all layers of the retina and CC in patients with JSLE without ocular involvement. Early screening and close follow-up were recommended. [ABSTRACT FROM AUTHOR]

Vitamin D, known for its essential role in calcium and bone homeostasis, has multiple effects beyond the skeleton, including regulation of immunity and modulation of autoimmune processes. Several reports have shown suboptimal serum 25 hydroxyvitamin D [25(OH)D] levels in people with different inflammatory and autoimmune rheumatic conditions, and an association between 25(OH)D levels, disease activity and outcomes. Although most available data pertain to adults, insights often are extended to children. Juvenile rheumatic diseases (JRDs) are a significant health problem during growth because of their complex pathogenesis, chronic nature, multisystemic involvement, and long-term consequences. So far, there is no definitive or clear evidence to confirm the preventive or therapeutic effect of vitamin D supplementation in JRDs, because results from randomized controlled trials (RCTs) have produced inconsistent outcomes. This review aims to explore and discuss the potential role of vitamin D in treating selected JRDs. Medline/PubMed, EMBASE, and Scopus were comprehensively searched in June 2023 for any study on vitamin D supplementary role in treating the most common JRDs. We used the following keywords: "vitamin D" combined with the terms "juvenile idiopathic arthritis", "juvenile systemic scleroderma", "juvenile systemic lupus erythematosus", "juvenile inflammatory myopathies", "Behcet disease", "periodic fever syndromes" and "juvenile rheumatic diseases". Observational studies have found that serum 25(OH)D concentrations are lower in juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile systemic scleroderma, Behcet disease and proinflammatory cytokine concentrations are higher. This suggests that vitamin D supplementation might be beneficial, however, current data are insufficient to confirm definitively the complementary role of vitamin D in the treatment of JRDs. Considering the high prevalence of vitamin D deficiency worldwide, children and adolescents should be encouraged to supplement vitamin D according to current recommendations. More interventional studies, especially well-designed RCTs, assessing the dose–response effect and adjuvant effect in specific diseases, are needed to determine the potential significance of vitamin D in JRDs treatment. [ABSTRACT FROM AUTHOR]

Background: Systemic lupus erythematosus (SLE) is a chronic, systemic, autoimmune disease of multifactorial origin. There are no previous studies in Colombia describing juvenile SLE (jSLE) prevalence, prompting this demographic description. Objective: This study aimed for prevalence calculation and epidemiologic analysis of jSLE) in Colombian patients, ages 0–19, from 2015 to 2019. Methods: This descriptive, cross-sectional study searched the Colombian Ministry of Health database for codes of the International Classification of Diseases, 10th revision (ICD-10) associated with jSLE to estimate the disease prevalence for the total population and for specific age groups at national and regional levels. Calculations used intercensal estimates of population based on the projections of the national statistics administrative department (DANE) from the most recent census. This paper presents a sociodemographic analysis of patients with jSLE. Results: The study identified in Colombia, from 2015 to 2019, 3680 cases with jSLE as the principal diagnosis. Calculated prevalence of jSLE was 25 cases per 100,000 population, with highest frequency in ages 15–19 and females (84%), with a female:male ratio of 5.1:1. Conclusion: Estimated prevalence of jSLE in Colombia is at the highest limit of worldwide findings. Consistent with reports in the literature, the disease involves females more frequently than males. [ABSTRACT FROM AUTHOR]

Introduction: Juvenile systemic lupus erythematosus (j-SLE) is a rare chronic autoimmune disease affecting multiple organs. Ranging from minor features, such as headache or mild cognitive impairment, to serious and life-threatening presentations, j-neuropsychiatric SLE (j-NPSLE) is a therapeutic challenge. Thus, the diagnosis of NPSLE remains difficult, especially in pediatrics, with no specific biomarker of the disease yet validated. Objectives: To identify central nervous system (CNS) disease biomarkers of j-NPSLE. Methods: A 5-year retrospective tertiary reference monocentric j-SLE study. A combination of standardized diagnostic criteria and multidisciplinary pediatric clinical expertise was combined to attribute NP involvement in the context of j-SLE. Neopterin and interferon-alpha (IFN-α) protein levels in cerebrospinal fluid (CSF) were assessed, together with routine biological and radiological investigations. Results: Among 51 patients with j-SLE included, 39% presented with j-NPSLE. J-NPSLE was diagnosed at onset of j-SLE in 65% of patients. No specific routine biological or radiological marker of j-NPSLE was identified. However, CSF neopterin levels were significantly higher in active j-NPSLE with CNS involvement than in j-SLE alone (p = 0.0008). Neopterin and IFN-α protein levels in CSF were significantly higher at diagnosis of j-NPSLE with CNS involvement than after resolution of NP features (respectively p = 0.0015 and p = 0.0010) upon immunosuppressive treatment in all patients tested (n = 10). Both biomarkers correlated strongly with each other (Rs = 0.832, p < 0.0001, n = 23 paired samples). Conclusion: CSF IFN-α and neopterin constitute promising biomarkers useful in the diagnosis and monitoring of activity in j-NPSLE. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate subclinical retinal microvascular changes with OCTA in juvenile systemic lupus erythematosus (JSLE) patients without ocular involvement. Ten eyes of 10 JSLE and 13 eyes of 13 age and sex-matched healthy controls (HCs) were enrolled. The superficial (SCP) and deep capillary plexus (DCP), FAZ parameters, the flow area of the outer retina, and choriocapillaris were evaluated using OCTA. All vessel density (VD) parameters in DCP were significantly lower in JSLE group compared to HCs. There was no significant difference between the groups in VD parameters of both SCP and ONH, FAZ, outer retina and choriocapillaris flow area. All DCP VD parameters showed a good ability to differentiate JSLE from HCs. OCTA could detect retinal vascular impairment in the deep macular region in JSLE patients with normal ocular examination. Furthermore, all DCP VD parameters have a good ability to discriminate JSLE from HCs. [ABSTRACT FROM AUTHOR]

BackgroundTo reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for juvenile proliferative lupus nephritis (LN). Induction immunosuppression CTPs outline treatment with either monthly intravenous (IV) cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in conjunction with one of three corticosteroid (steroid) CTPs: primarily oral, primarily IV or mixed oral/IV. The acceptability and in-practice use of these CTPs are unknown. Therefore, the primary aims of the pilot study were to demonstrate feasibility of adhering to the LN CTPs and delineate barriers to implementation in clinical care in the US. Further, we aimed to explore the safety and effectiveness of the treatments for induction therapy.MethodsForty-one patients were enrolled from 10 CARRA sites. Patients had new-onset biopsy proven ISN/RPS class III or IV proliferative LN, were starting induction therapy with MMF or IV CYC and high-dose steroids and were followed for up to 24 months. Routine clinical data were collected at each visit. Provider reasons for CTP selection were assessed at baseline. Adherence to the CTPs was evaluated by provider survey and medication logs. Complete and partial renal responses were reported at 6 months.ResultsThe majority of patients were female (83%) with a mean age of 14.7 years, SD 2.8. CYC was used more commonly than MMF for patients with ISN/RPS class IV LN (vs. class III), those who had hematuria, and those with adherence concerns. Overall adherence to the immunosuppression induction CTPs was acceptable with a majority of patients receiving the target MMF (86%) or CYC (63%) dose. However, adherence to the steroid CTPs was poor (37%) with large variability in dosing. Renal response endpoints were exploratory and did not show a significant difference between CYC and MMF.ConclusionsOverall, the immunosuppression CTPs were followed as intended in the majority of patients however, adherence to the steroid CTPs was poor indicating revision is necessary. In addition, our pilot study revealed several sources of treatment selection bias that will need to be addressed in for future comparative effectiveness research.

Purpose: To evaluate meibomian gland loss and its possible association with disease duration and activity in Juvenile Systemic Lupus Erythematosus (JSLE) patients' without dry eye symptoms or signs. Study design: Prospective clinical study. Methods: Ten eyes of 10 JSLE patients were evaluated using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and 14 eyes of 14 healthy controls were also enrolled. Ocular Surface Disease Index (OSDI) questionnaire and Schirmer I test were performed. Lid margin score, meibum quality and expressibility scores were evaluated. Noninvasive first breakup time (NIF-BUT) and noninvasive average breakup time (NIAvg-BUT), meibomian gland dropout area (MGDA) and meibography scores were evaluated using non-contact meibography (Sirius; CSO). Results: There was no significant difference between the JSLE patients and the healthy controls in the OSDI, NIF-BUT, NIAvg-BUT and Schirmer I tests. Also lid margin score, meibum quality and expressibility scores were not significantly different between the groups. However, JSLE patients had increased upper and lower lid MGDA and increased upper lid, lower lid and total meibography scores compared to the healthy subjects. Furthermore, the duration of JSLE showed a high positive correlation with upper and lower lid MGDA and meibography scores. Conclusion: Meibomian gland loss could be observed in JSLE patients with no clinical signs and symptoms of dry eye. Considering the longer life expectancy of children, the positive correlation of disease duration with the meibomian gland reveals the importance of routine eye examinations from the diagnosis of the disease. [ABSTRACT FROM AUTHOR]

Juvenile systemic lupus erythematosus (JSLE) is diagnosed in children younger than 18 years of age. Depression and anxiety are common, but not well understood in JSLE. We investigated the clinical and psychological factors associated with the psychological manifestations of JSLE. Twenty-nine JSLE patients were recruited for the study. Patients completed surveys evaluating their psychological status and perceptions about their health. Medical records were used to obtain laboratory results. The JSLE patient population was compared with adult-onset SLE (ASLE) patients and unaffected controls. Kidney involvement was associated with depression in the JSLE patients. The BUN levels, BUN/creatinine ratio, and leukocyturia were all significantly associated with depressive symptoms. Multivariate analysis found that the BUN/creatinine ratio was the most predictive value for both depression and anxiety. Depressive symptoms in JSLE were less pronounced than in ASLE, although anxiety was not different. Age and education are likely to be protective against depression in the JSLE patients. These findings may indicate that symptomatology is an important indicator of whether the patient needs psychiatric care.

Left ventricular noncompaction (LVNC) is a structural abnormality of the left ventricular myocardium of unknown cause. Cardiovascular system involvement is an important manifestation in juvenile systemic lupus erythematosus (jSLE) and is a leading cause of morbidity and mortality. To present a case of jSLE with LVNC and Hashimoto's thyroiditis. A 15-year-old Tunisian girl with a history of Hashimoto's thyroiditis presented with paroxysmal chest pain, exertional dyspnea, headache, and polyarthralgia and was diagnosed with jSLE. Clinical examination revealed a febrile patient (39 °C). There was no tachypnea, no evidence of right heart failure, no audible murmurs on cardiac auscultation, and no edema of the lower limbs. Laboratory investigations revealed leucopenia (3500/mm3), anemia (10 g/dl), elevated erythrocyte sedimentation rate (ESR)(50 mm\1sth), C-reactive protein (20 mg/l). Infections were excluded with multiple negative blood cultures and negative anti-streptolysin O antibodies. The antinuclear antibody (ANA) was positive homogenous at 1/10,000 and both anti-native DNA and anti-SSA were positive. The electrocardiogram (ECG) showed sinus tachycardia, the transthoracic echocardiography revealed pericarditis associated with a non-compacted LV and a small pericardial effusion. Thus, a cardiac magnetic resonance imaging (CMRI) was performed confirming LVNC with a hypertrabeculated sub-endocardium. There was no proteinuria, hypocomplementemia or cerebral vasculitis on cerebral magnetic resonance imaging (MRI). The patient received high-dose corticosteroids associated with beta-blocker therapy with a favorable outcome. A rare case of LVNC which was accidentally discovered in a patient with jSLE and Hashimoto's thyroiditis is documented. Follow-up is needed for the development possible complications. [ABSTRACT FROM AUTHOR]

Background: Juvenile systemic lupus erythematosus (jSLE) is known to be more severe and with a higher frequency of renal and central nervous system impairment when compared to systemic lupus erythematosus in adults. The study of immunological characteristics of jSLE patients might help to envisage better treatment strategies to reduce the burden of the disease. Objective: To characterize peripheral lymphocytes, assessing activation markers, and PD-1 expression on T cells; to evaluate in vitro cytokine expression upon stimulation in jSLE patients and age-matched controls. Methodology: Eighteen jSLE patients on low disease activity and 25 matched healthy adolescents were evaluated for immune activation and PD-1 expression on peripheral blood lymphocytes by flow cytometry. Twenty-one cytokines were assessed by X-MAP technology after in vitro stimulation of peripheral blood with phytohemagglutinin. Results: jSLE patients had lower numbers of CD4 T, CD8 T, B, and NK cells; higher central memory CD8 T cell percentages were noted in jSLE adolescents in comparison with controls (p = 0.014). B cells subsets showed a higher percentage of exhausted memory subset than controls (p = 0.014). The expression of PD-1 on CD4 T and CD8 T cells did not show relevant changes in jSLE adolescents. After stimulation of peripheral blood, cell supernatant of jSLE patients showed a trend to lower concentrations of IL-10 (p =0.080) and higher concentrations of IL-23 (p = 0.063) than controls. Conclusions: jSLE patients on low disease activity maintain lymphopenia of all subsets, with a B cell profile of exhaustion. Upon in vitro stimulation, peripheral blood cell supernatant showed a shift to IL-23, suggesting a role of inhibitors of this cytokine as another potential therapeutic target for those patients. [ABSTRACT FROM AUTHOR]

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Objective: Demographics, clinical features, and biomarkers do not consistently anticipate risk of end-stage renal disease (ESRD) in juvenile lupus nephritis (LN). Here, the existence of autoantibody clusters predictive of ESRD was explored in a cohort of biopsy-proven juvenile LN. Methods: A retrospective chart review was performed of patients with juvenile systemic lupus erythematosus (jSLE) and biopsy-confirmed LN. Primary endpoints were ESRD and mortality. Patients were included for K-medians cluster analysis if they had a complete autoantibody profile, which included ANA titer, anti-dsDNA, anti-Smith, anti-RNP, anti-Ro/SSA, anti-La/SSB. Chi-square test was used for categorical variables and one-way ANOVA for continuous measures. Significance was p<0.05. Results: Fifty-three met inclusion criteria, of which 45 were female and 37 were black. Over 80% developed LN within one year of jSLE onset and more than half (n=29) had LN at diagnosis of jSLE. Six developed ESRD. No mortalities were reported. Forty-six had a complete autoantibody profile, including four with ESRD. Three clusters were identified. Group 1 (n=8) was defined by anti-dsDNA; group 2 (n=28) by high-titer ANA (>1:1280), anti-Smith, anti-RNP, and anti-Ro/SSA; and group 3 (n=10) by anti-dsDNA and anti-Ro/SSA. There was no difference between the groups in demographics, jSLE manifestations, or markers of renal function. One in group 2 and three in group 3 developed ESRD. Those in group 3 were younger at diagnosis of LN (p=0.084) and had the highest frequency of ESRD (p=0.025). Conclusion: Cluster analysis revealed the highest frequency of ESRD in the group with LN defined by anti-Ro/SSA and anti-dsDNA co-positivity. Key Points • Lupus nephritis commonly is present at diagnosis of juvenile systemic lupus erythematosus or develops within the first year. • End-stage renal disease was more frequent in the cluster defined by anti-dsDNA and anti-Ro/SSA co-positivity; patients with this profile may benefit from more aggressive immunosuppression. [ABSTRACT FROM AUTHOR]

Abstract Background Consensus treatment plans have been developed for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in childhood-onset systemic lupus erythematosus. However, patients who do not respond to initial therapy, or who develop renal flare after remission, warrant escalation of treatment. Our objective was to assess current practices of pediatric nephrologists and rheumatologists in North America in treatment of refractory proliferative LN and flare. Methods Members of Childhood Arthritis and Rheumatology Research Alliance (CARRA) and the American Society for Pediatric Nephrology (ASPN) were surveyed in November 2015 to assess therapy choices (other than modifying steroid dosing) and level of agreement between rheumatologists and nephrologists for proliferative LN patients. Two cases were presented: (1) refractory disease after induction treatment with corticosteroid and cyclophosphamide (CYC) and (2) nephritis flare after initial response to treatment. Survey respondents chose treatments for three follow up scenarios for each case that varied by severity of presentation. Treatment options included CYC, mycophenolate mofetil (MMF), rituximab (RTX), and others, alone or in combination. Results Seventy-six respondents from ASPN and foty-one respondents from CARRA represented approximately 15 % of the eligible members from each organization. Treatment choices between nephrologists and rheumatologists were highly variable and received greater than 50 % agreement for an individual treatment choice in only the following 2 of 6 follow up scenarios: 59 % of nephrologists, but only 38 % of rheumatologists, chose increasing dose of MMF in the case of LN refractory to induction therapy with proteinuria, hematuria, and improved serum creatinine. In a follow up scenario showing severe renal flare after achieving remission with induction therapy, 58 % of rheumatologists chose CYC and RTX combination therapy, whereas the top choice for nephrologists (43 %) was CYC alone. Rheumatologists in comparison to nephrologists chose more therapy options that contained RTX in all follow up scenarios except one (p

Introduction: The aim of the present study was to investigate serum phosphate and uric acid levels (UA) in juvenile systemic lupus erythematosus (jSLE) and to ascertain their relationship, if any, with disease activity and lupus nephritis. Methods: Included in the study were 18 children with jSLE who were investigated retrospectively during the active phase (AP) and remission phase (RP) of jSLE. Results: Serum phosphate and UA levels were found to be significantly lower in AP than in the RP (2.5±0.3/4.2±0.08 mg/dL, p=0.0001 and 2.9±0.3/5±0.2 mg/dL, p=0.0001, respectively). Serum phosphate levels were strongly correlated with serum albumin levels (r=0.772, p=0.0001) and platelet count (r=0.735, p=0.001) and negative associated with D-dimer (r=−0.750, p=0.0001) in AP. No correlation was identified between serum phosphate and UA or creatinine levels in patients with AP, while serum phosphate and UA levels were similar in children with and without nephritis (p>0.05). Serum phosphate levels were correlated with platelet count and UA levels and were inversely related with D-dimer levels in jSLE patients with nephritis (p<0.05). Discussion and Conclusion: Serum phosphate and UA levels can reflect the activation and low serum levels may be useful biomarkers for the detection of AP in jSLE patients with and without nephritis. [ABSTRACT FROM AUTHOR]

Objective: Anemia is common in patients with juvenile systemic lupus erythematosus (jSLE). While autoimmune hemolytic anemia (AIHA) is the only etiology included in the classification criteria, the etiology of anemia in jSLE may be diverse. We aimed to investigate the etiology of anemia in jSLE and the relationship between anemia and disease characteristics at onset and during the follow-up period. Methods: Patients diagnosed with jSLE who met the Systemic Lupus Erythematosus International Collaborating Clinics classification criteria between January 2012 and December 2020 were retrospectively analyzed. Results: Hematologic involvement was observed in 70% of the patients. Anemia was the most common cytopenia among patients (60%). Anemia of chronic disease (ACD) and AIHA were the most common etiological factors, both observed in 23% of patients. Patients with anemia had a significantly higher rate of positive ds-DNA antibody and higher erythrocyte sedimentation rate (ESH) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores. ESH, serum ferritin, and SLEDAI scores negatively correlated with hemoglobin levels in patients with anemia. Iron deficiency was the sole etiology of new-onset anemia. Patients with new-onset anemia during the follow-up period had significantly lower hemoglobin values at onset and a higher rate of renal involvement. Conclusion: Anemia in jSLE is mostly AIHA and ACD, but iron deficiency is not rare. The severity of inflammation is associated with the severity of anemia. During the follow-up period, iron deficiency was the predominant cause of anemia, especially in patients with lower hemoglobin concentrations at onset and renal involvement. [ABSTRACT FROM AUTHOR]

Mohammed Abd El Monem Teama,1 Marwa Adham El-Mohamdy,2 Fatma Abdellah Abdullah Mahmoud,2 Fatma Mohammed Badr1 1Internal Medicine Department, Division of Rheumatology and Immunology, Faculty of Medicine, Ain Shams University, Cairo, Egypt; 2Clinical Pathology Department, Faculty of medicine, Ain Shams University, Cairo, EgyptCorrespondence: Mohammed Abd El Monem Teama 2 Staff Member Buildings of Ain Shams University, El Al Waili/El-Abaseya, Cairo, 11517, EgyptTel +20 1277580006Email mohteama2009@yahoo.comObjective: This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile systemic lupus erythematosus (JSLE) patients and their association with different clinical manifestations and disease activity.Patients and Methods: A cross-sectional study that includes 100 JSLE patients from Ain Shams University Hospital was conducted. All subjects underwent history taking, clinical examination, assessment of disease activity based on the SLE disease activity index (SLEDAI), laboratory investigations, and tests for autoantibodies, namely ANA, anti-dsDNA, and anti-ENA antibodies, including anti-Ro (SSA), anti-La (SSB), anti-Smith (Sm), and anti-U1-ribonucleoprotein (U1-RNP).Results: The most common clinical features were polyarthralgia (71%), haematological manifestations (65%), malar rash (54%), and nephritis (51%), respectively. All patients had positive ANA (100%), while anti-dsDNA frequency was 83%. The most common anti-ENA antibodies were anti-RNP (41%), anti-Sm (31%), anti-SSA (27%), and anti-SSB (20%), respectively. Anti-RNP had a clinical association with oral ulcer, Raynaud’ phenomena, haematological, neuropsychiatric and thromboembolic manifestations. Meanwhile, anti-Sm had a significant association with serositis, mucocutaneous, constitutional, and neuropsychiatric manifestations. Anti-SSA was associated with mucocutaneous, musculoskeletal, Raynaud’ phenomena, renal, haematological and cardiac manifestations, while anti-SSB was significantly associated with malar rash, serositis, thromboembolic, musculoskeletal, and neuropsychiatric manifestations. Concerning SLEADI score, anti-dsDNA antibody was significantly associated with moderate disease activity score (p=0.032) while anti-SSA significantly associated with high disease activity (p=0.045). Both anti-SSB and anti-Sm were significantly associated with both moderate and high disease activities, meanwhile anti-U1-RNP was associated with moderate disease activity (p=0.014).Conclusion: Anti-dsDNA and anti-ENAs antibodies were frequently found in JSLE patients (83%, 63%), respectively. They were significantly associated with variable clinical manifestations and could be used as predictors for assessment of disease activity.Keywords: juvenile systemic lupus erythematosus, clinical manifestations, anti-ENA, disease activity score

Pulmonary involvement in children with connective tissue systemic diseases is rare pathology that, however, has significant impact on treatment strategy and prognosis. This article presents data on the pulmonary involvement incidence, development, clinical features and results of visualization methods in children with such connective tissue systemic diseases as systemic sclerosis, juvenile dermatomyositis, systemic lupus erythematosus, and Sjogren’s syndrome. The issues of pathogenesis, risk factors of lung damage, therapeutic approaches and prognosis are considered.

Objectives: To describe the care provided by Brazilian nurses to patients with Juvenile Systemic Lupus Erythematosus (JSLE) using a self-administered questionnaire and to evaluate the possible association of demographic data and knowledge of the disease with availability of tools for diagnosis and treatment in Brazilian pediatric services. Method: This is a cross-sectional observational descriptive study that analyzed the care provided by Brazilian nurses to patients with JSLE based on the answers to a self-administered questionnaire. Questions included demographics of professionals, nursing consultation, usage of tools to assess disease activity, diagnostic criteria, general care, available practices, transition program, and practices needed for better care. Results: A total of 111/373 (29.4%) questionnaires were completed, most professionals were female (90.1%). Hospitalization (45.9%) and poor medication adherence (27.9%) were the most reported problems, and lack of knowledge about the institutional transition process to adult care was mentioned by half of the professionals (50.5%). Comparisons of professionals on expected care of patients regarding the type of service, hospitalized patient care, and care provided to patients with JSLE did not reveal statistically significant differences (p > 0.05). Nurses who care for patients with JSLE are more likely to work in a public setting (14.5% vs. 36.8%, p = 0.048) and less likely to verify vaccination records (0.12.9% vs. 36.8%, p = 0.038). Nurses who worked in the inpatient setting with patients with JSLE were less likely to address pain concerns (11.7% vs. 61.6%, p < 0.0001) compared to nurses who didn't work with JSLE patients. However, they were more likely to discuss sunscreen use (54.9% vs. 25%, p < 0.05) and be part of a multi-disciplinary team (84.3 vs. 50%, p < 0.0001). Conclusions: This study demonstrates that the care provided by nurses to patients with JSLE does not consider all the resources available to assess the disease and occurs differently in the inpatient versus outpatient settings. Future research should address this gap in care. Lack of JSLE awareness was commonly reported among professionals, confirming the need for greater knowledge about JSLE, its treatment, and ways to improve adherence. [ABSTRACT FROM AUTHOR]

Juvenile systemic lupus erythematosus (JSLE) is diagnosed in children younger than 18 years of age. Depression and anxiety are common, but not well understood in JSLE. We investigated the clinical and psychological factors associated with the psychological manifestations of JSLE. Twenty-nine JSLE patients were recruited for the study. Patients completed surveys evaluating their psychological status and perceptions about their health. Medical records were used to obtain laboratory results. The JSLE patient population was compared with adult-onset SLE (ASLE) patients and unaffected controls. Kidney involvement was associated with depression in the JSLE patients. The BUN levels, BUN/creatinine ratio, and leukocyturia were all significantly associated with depressive symptoms. Multivariate analysis found that the BUN/creatinine ratio was the most predictive value for both depression and anxiety. Depressive symptoms in JSLE were less pronounced than in ASLE, although anxiety was not different. Age and education are likely to be protective against depression in the JSLE patients. These findings may indicate that symptomatology is an important indicator of whether the patient needs psychiatric care. [ABSTRACT FROM AUTHOR]

Objective: To evaluate how chronic gingivitis treatment impacts the oral and circulating cytokine expressions after six‐month follow‐up in patients with juvenile systemic lupus erythematosus (jSLE) and also to evaluate the circulating expression of anti‐Porphyromonas gingivalis peptidylarginine deiminase antibodies (anti‐PPAD) before and after treatment. Background: Juvenile systemic lupus erythematosus patients present a worse periodontal condition associated with higher gingival crevicular fluid (GCF) levels of interleukin (IL)‐1β, IL‐8, granulocyte colony‐stimulating factor (G‐CSF), interferon‐γ and monocyte chemoattractant protein (MCP)‐1. Materials and Methods: Twenty‐one adolescents with jSLE (mean age: 16.2 ± 1.5 years) were recruited. Participants were rheumatologically and periodontally examined. All individuals were clinically diagnosed with gingival inflammation. Chronic gingivitis treatment consisted of supragingival scaling, prophylaxis and oral hygiene instructions. The cytokine levels were determined by bead‐based multiplex assays and the anti‐PPAD levels by ELISA. Gingival crevicular fluid (GCF) and serum samples were collected at baseline and 6 months after treatment. Results: We observed a reduction in attachment loss, SLE Disease Activity Index (SLEDAI), IL‐1β, IL‐10 and MCP‐1 GCF levels, and the IL‐4 and IL‐5 serum levels 6 months after periodontal treatment. On the contrary, a significant increase in GCF expression of IL‐4, IL‐12, IL‐17, IFN‐γ and serum levels of anti‐PPAD antibody was observed. Conclusion: Juvenile systemic lupus erythematosus patients seem to positively benefit from periodontal treatment by a significantly reduced CAL, a GCF reduction of pro‐inflammatory cytokines and an increasing of anti‐inflammatory ones. However, an increase in the GCF expression of IL‐17 and the serum expression of anti‐PPAD antibody 6 months after periodontal treatment might negatively affect the treatment outcome of such patients in the long term. [ABSTRACT FROM AUTHOR]

Objectives The European League Against Rheumatism and American College of Rheumatology 2019 (EULAR/ACR-19) criteria for the diagnosis of SLE were recently published, with the stated goal of maintaining the level of sensitivity and raising the level of specificity for classification of SLE in adults. The aim of this study is to examine their application to juvenile SLE (jSLE) patients. Methods In this multicentre study the charts of jSLE patients from three tertiary medical centres were reviewed and compared with patients with non-jSLE diagnosis. Paediatric rheumatologists, blinded to the original diagnosis, reviewed and diagnosed all cases. Paediatric patients' clinical and laboratory data were retrospectively extracted and then examined with regard to how they met the new and old criteria. Results Included were 225 patients (112 jSLE, 113 non-SLE). When applied to juvenile SLE classification, the sensitivity of the new EULAR/ACR-19 criteria was 0.96 (95% CI: 0.9, 0.99) and the specificity was 0.89 (95% CI: 0.82, 0.94). These were comparable to the SLICC criteria. The sensitivity of the EULAR/ACR-19 criteria improves over time and was 0.83 12 months following disease onset, reaching 0.96 after longer than 24 months. Conclusion Among a cohort of jSLE patients, sensitivity of the new EULAR/ACR-19 criteria was found to be high and specificity may have improved slightly compared with the SLICC-12 criteria. We support the use of the new classification criteria for paediatric patients in future jSLE studies, but it should be noted that its specificity is lower than for adults. [ABSTRACT FROM AUTHOR]

Amaç: Avasküler nekrozis (AVN), çocukluk çağı başlangıçlı sistemik lupus eritematozusun (jSLE) yıkıcı bir komplikasyonudur. Tek merkezden yapılan bu çalışmanın amacı jSLE hastalarında AVN sıklığını ve bu hastaların klinik özelliklerini belirlemektir. Gereç ve Yöntem: Amerikan Romatoloji Koleji’nin sınıflama kriterlerine göre tanı almış 58 jSLE hastası bu geriye dönük çalışmaya dahil edildi. jSLE hastalarının hastalık aktiviteleri SLE hastalık aktivite indeksi baz alınarak (SLEDAI-2K) ölçüldü. Organ hasarı Amerikan Romatoloji Koleji’nin SLE hastalarında uyguladığı hasar indeksinin çocukluklardaki versiyonu kullanılarak son muayenede belirlendi. Bulgular: Elli sekiz jSLE hastasının %86,2 (50)’si kız hastaydı. Ortalama bazal SLEDAI-2K skoru 21,47±8,96 idi. En az bir organda hasarı olan hasta sayısı 24 (%41,4) idi, bunlardan 5 tanesinde (%8,6) AVN vardı. AVN olan ve olmayan hastalar arasında çeşitli değikenlere göre istatistiksel anlamlı bir farklılık saptanmadı. Sadece bazal kompleman 3 (C3) düzeyi AVN hastalarında, AVN olmayan hastalara göre anlamlı olarak düşüktü. Sonuç: Bazal düşük C3 düzeyi jSLE hastalarında AVN gelişimini tahmin etmede ön görücü olabilir. jSLE hastalarında AVN gelişimi için muhtemel risk faktörlerini inceleyen çok merkezli çalışmalara ihtiyaç vardır.

Background: Consensus treatment plans have been developed for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in childhood-onset systemic lupus erythematosus. However, patients who do not respond to initial therapy, or who develop renal flare after remission, warrant escalation of treatment. Our objective was to assess current practices of pediatric nephrologists and rheumatologists in North America in treatment of refractory proliferative LN and flare. Methods: Members of Childhood Arthritis and Rheumatology Research Alliance (CARRA) and the American Society for Pediatric Nephrology (ASPN) were surveyed in November 2015 to assess therapy choices (other than modifying steroid dosing) and level of agreement between rheumatologists and nephrologists for proliferative LN patients. Two cases were presented: (1) refractory disease after induction treatment with corticosteroid and cyclophosphamide (CYC) and (2) nephritis flare after initial response to treatment. Survey respondents chose treatments for three follow up scenarios for each case that varied by severity of presentation. Treatment options included CYC, mycophenolate mofetil (MMF), rituximab (RTX), and others, alone or in combination. Results: Seventy-six respondents from ASPN and foty-one respondents from CARRA represented approximately 15 % of the eligible members from each organization. Treatment choices between nephrologists and rheumatologists were highly variable and received greater than 50 % agreement for an individual treatment choice in only the following 2 of 6 follow up scenarios: 59 % of nephrologists, but only 38 % of rheumatologists, chose increasing dose of MMF in the case of LN refractory to induction therapy with proteinuria, hematuria, and improved serum creatinine. In a follow up scenario showing severe renal flare after achieving remission with induction therapy, 58 % of rheumatologists chose CYC and RTX combination therapy, whereas the top choice for nephrologists (43 %) was CYC alone. Rheumatologists in comparison to nephrologists chose more therapy options that contained RTX in all follow up scenarios except one (p < 0.05). Conclusions: Therapy choices for pediatric rheumatologists and nephrologists in the treatment of refractory LN or LN flare were highly variable with rheumatologists more often choosing rituximab. Further investigation is necessary to delineate the reasons behind this finding. This study highlights the importance of collaborative efforts in developing consensus treatment plans for pediatric LN. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to assess the urinary soluble alpha chain of the interleukin-2 receptor (sCD25) concentrations in patients with juvenile systemic lupus erythematosus (JSLE) and to evaluate its validity to be a possible marker of disease activity in patients with lupus nephritis (LN). Patients and methods: We assessed sCD25 concentrations in urine samples obtained from 53 JSLE patients (15 males, 38 females; median age 11 years; range, 7 to 17 years) and 30 age- and sex-matched apparently healthy controls (10 males, 20 females; median age 10 years; range, 6 to 16 years). Concentrations were normalized according to urinary creatinine excretion. JSLE patients were subjected to clinical examination and assessment of overall disease activity by Systemic Lupus Erythematous Disease Activity Index (SLEDAI), while evaluation of LN activity was preformed using Systemic Lupus International Collaborating Clinics (SLICC) renal activity score. Results: The JSLE patients had significantly higher normalized urinary sCD25 concentrations compared to the healthy controls (p=0.001). Patients with active LN had significantly higher normalized urinary sCD25 levels than active JSLE patients without LN (p=0.002) and JSLE patients with inactive disease (p<0.001). A significant positive correlation was found between normalized urinary sCD25 concentrations with different activity parameters such as proteinuria (p=0.004), SLEDAI (p<0.001), renal SLEDAI (p<0.001), and SLICC renal activity score (p<0.001). A significant negative correlation was found between urinary sCD25 and complement 3 (p<0.001). Conclusion: Urinary concentrations of sCD25 were significantly elevated in JSLE patients, particularly in those with active LN. The remarkable association between urinary sCD25 concentrations and different renal disease activity parameters implies that urinary sCD25 can be a beneficial marker to monitor active nephritis in JSLE patients. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease, characterized by circulating autoantibodies, widespread inflammation, and tissue destruction. The association between cancer and SLE is intriguing in view of their common pathophysiological pathways. It is established that there is an increased risk for overall malignancies in SLE, with standardized incidence ratios ranging from 1.14 to 3.6. Hematological malignancies are more common than solid organ malignancies in SLE. Malignancy when associated with lupus usually follows the diagnosis, but can occur concurrently or even predate the diagnosis of SLE. Neoplasms of dendritic cells (DCs), the key antigen‑presenting cells, are rare, and they account for <1% of all lymph node tumors. Interdigitating DC sarcoma (IDCS) is a rare hematological malignancy of DCs, with only a few over 100 cases being reported in literature. IDCS is usually seen in elderly males in their sixth decade. Here, we report a rare case of SLE and IDCS occurring together in a 5‑year‑old girl. The persistence of lymphadenopathy despite improvement in other lupus‑specific features following the initiation of treatment led to a suspicion of malignancy which was proven on histopathology. Another unique feature of our case was that remission and relapse of both SLE and IDCS occurred synchronously. Although this presentation is rare, clinicians must be aware of the possibility of SLE and malignancies occurring together, especially in the presence of atypical clinical manifestations such as persistent lymphadenopathy. This knowledge helps in early diagnosis and effective treatment of both these conditions. [ABSTRACT FROM AUTHOR]

Cardiac involvement in systemic lupus erythematosus (SLE) is well documented. The pericardium, myocardium and endocardium, as well as the coronary arteries, the valves and the conduction system can all be affected. While pericarditis is common, arrythmias are less frequently described. We present a 13-year-old male, who had fatigue, anorexia, weight loss, myalgias and arthralgias for four months. On physical examination, we identified bradycardia (heart rate 31–50 bpm), oral and nasal ulcers and polyarthritis. The laboratory results showed hemolytic anemia, hypocomplementemia, antinuclear and anti-dsDNA antibodies, hematuria and non-nephrotic proteinuria. Renal function was normal. Lupus nephritis class II was diagnosed by kidney biopsy. On the transthoracic echocardiogram we identified a minimal pericardial effusion, suggesting pericarditis, and, on the electrocardiogram, we detected sinus arrest with junctional rhythm, denoting sinus node dysfunction. The patient was diagnosed with juvenile SLE with cardiac, renal, musculoskeletal and hematologic involvement. Disease remission and cardiac rhythm control were obtained with steroids and mycophenolate mofetil. Currently, the patient is asymptomatic, with normal sinus rhythm. We described an adolescent with SLE who had sinus node dysfunction upon diagnosis. Other cases have been reported in adults but none in juvenile SLE. All SLE patients should have a thorough cardiac examination to promptly diagnose and treat the innumerous cardiac manifestations of this disease. [ABSTRACT FROM AUTHOR]

Background: Reports on vaccine responses in immunocompromised patients, such as juvenile systemic lupus erythematosus (jSLE), have shown highly variable results. Objective: To compare the immune response and safety after a Tdap booster in 26 jSLE patients and 26 matched healthy adolescents. Methodology: Adverse events and disease activity were evaluated. Lymphocyte immunophenotyping was performed by flow cytometry. Tetanus, diphtheria and pertussis toxin antibodies were assessed by ELISA; whole blood was stimulated with whole cell pertussis and supernatants were assessed for cytokines by xMAP. Results: Both groups showed a similar frequency of adverse events. There was no evidence of disease reactivation after Tdap booster in the jSLE cohort. Both groups showed a significant increase in antibody titers for all three antigens on D14 and D28 (p < 0.001). jSLE patients had a significantly lower increase in diphtheria titers than the control group (p = 0.007). jSLE patients had a distinct titer increase of tetanus and pertussis antibodies when compared to controls (p = 0.004 and p < 0.001, respectively). There was a lower frequency of pertussis seroconversion in the jSLE group on D14 (p = 0.009), D28 (p = 0.023), D12m (p = 0.015) and D24m (p = 0.004). Cellular immune response to Bordetella pertussis showed significantly lower levels of IFNγ (p < 0.001) and higher levels of IL10, IL12, IL21 and TNFα in jSLE patients than controls. Conclusions: jSLE patients had good response to Tdap booster dose for the tetanus antigen, but not for diphtheria and pertussis. This vaccine was safe in relation to adverse events and absence of disease reactivation. [ABSTRACT FROM AUTHOR]

Objective: The purpose of the present study was to investigate the prevalence and incidence of juvenile systemic lupus erythematosus (JSLE) in Korea.Methods: The data were collected from the National Health Insurance Claims Database of Korea. JSLE was identified using the diagnostic code M32 from the Korean Standard Classification of Diseases. Patients between 5 and 18 years old, who had at least 1 claim for JSLE from January 1, 2016, to December 31, 2017, as final diagnosis, were analyzed in the study. For prevalent cases, patients who used, at least 1 time, any type of medical services with a diagnostic code of M32 were selected. For incident cases, patients who did not use medical services with the M32 code 1 year prior and who were newly registered in 2017 were defined. Change-point analysis was used to find the age at which changes in prevalence and incidence occurred.Results: The prevalence of JSLE was 6.92 per 100,000 persons and the incidence of JSLE was 2.76 per 100,000 person-years in patients between 5 and 18 years old. The prevalence and incidence of JSLE were higher in females than in males. According to the change-point analysis, we found that the incidence and prevalence of female patients increased rapidly at the ages of 14 and 15 years, respectively.Conclusion: This Korean population-based epidemiological study of JSLE showed similar epidemiologic profiles to Asian population in other studies. The distribution of age, ethnicity, and pubertal status are important factors that influence population estimates of JSLE incidence and prevalence. [ABSTRACT FROM AUTHOR]

Background: Juvenile systemic lupus erythematosus (JSLE) is usually associated with vitamin D deficiency and low bone mineral density. Objectives: To evaluate serum levels of 25-OH vitamin D in JSLE patients and to correlate these findings with disease activity and bone density. Methods: This study was conducted on 100 patients with JSLE and 100 healthy children as controls. Disease duration and SLEDAI for disease activity were evaluated. CBC, anti-dsDNA, C3,C4,24hr urinary proteins, creatinine, estimated glomerular filtration rate(e-GFR),Ca,P,PTH, 25 (OH) D levels, and bone mineral density(BMD)Z score were measured. Results: There were significant differences in mean 25(OH)D concentration between patients group (19.37 ± 9.72 ng/ml) and controls 35.90 ± 9.66 ng/ml(p < 0.05), with significant difference between active and inactive patients (p < 0.05).There were significant negative correlations between serum 25(OH)D and SLEDAI (r-0.545, p 0.001), steroid dose (r-0.561, p 0.001), anti-dsDNA (r-0.685, p 0.006), 24 hr-proteinuria (r-0.738, p 0.001) and PTH (r-0.335, p 0.001), significant positive correlations between 25(OH)D and C3 (r0.617, p 0.001),C4 (r0.544, p 0.001) serum Ca (r0.424, p 0.001) and Z score (r0.561, p 0.001),with non-significant correlations between 25(OH)D and serum P and both disease & steroid duration, (p > 0.05). Conclusion: Vitamin D deficiency is common in JSLE, it's correlated significantly with disease activity and bone mineral density. [ABSTRACT FROM AUTHOR]

Rationale: Kawasaki disease (KD) is an acute vasculitis of small and medium vessels; whereas systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease. Their presentation is varied and not always straightforward, leading to misdiagnosis. There have been case reports of lupus onset mimicking KD and KD presenting as lupus-like. Coexistence of both diseases is also possible.Patient concerns: We present three adolescents, one with fever, rash, arthritis, nephritis, lymphopenia, and coronary aneurysms, a second patient with rash, fever, aseptic meningitis, and seizures, and a third patient with fever, rash, and pleural effusion.Diagnoses: The first patient was finally diagnosed with SLE and KD, the second patient initially diagnosed as KD but eventually SLE and the third patient was diagnosed at onset as lupus but finally diagnosed as KD.Interventions: The first patient was treated with IVIG, corticosteroids, aspirin, coumadin and mycophenolate mofetil. The second patient was treated with IVIG, corticosteroids and methotrexate and the third patient with IVIG, aspirin and corticosteroids.Lessons: Both diseases may mimic each other's clinical presentation. KD in adolescence presents with atypical signs, incomplete presentation, and develop coronary complications more commonly. An adolescent with fever and rash should include KD and SLE in the differential diagnosis.

Abstract Background To reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for juvenile proliferative lupus nephritis (LN). Induction immunosuppression CTPs outline treatment with either monthly intravenous (IV) cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in conjunction with one of three corticosteroid (steroid) CTPs: primarily oral, primarily IV or mixed oral/IV. The acceptability and in-practice use of these CTPs are unknown. Therefore, the primary aims of the pilot study were to demonstrate feasibility of adhering to the LN CTPs and delineate barriers to implementation in clinical care in the US. Further, we aimed to explore the safety and effectiveness of the treatments for induction therapy. Methods Forty-one patients were enrolled from 10 CARRA sites. Patients had new-onset biopsy proven ISN/RPS class III or IV proliferative LN, were starting induction therapy with MMF or IV CYC and high-dose steroids and were followed for up to 24 months. Routine clinical data were collected at each visit. Provider reasons for CTP selection were assessed at baseline. Adherence to the CTPs was evaluated by provider survey and medication logs. Complete and partial renal responses were reported at 6 months. Results The majority of patients were female (83%) with a mean age of 14.7 years, SD 2.8. CYC was used more commonly than MMF for patients with ISN/RPS class IV LN (vs. class III), those who had hematuria, and those with adherence concerns. Overall adherence to the immunosuppression induction CTPs was acceptable with a majority of patients receiving the target MMF (86%) or CYC (63%) dose. However, adherence to the steroid CTPs was poor (37%) with large variability in dosing. Renal response endpoints were exploratory and did not show a significant difference between CYC and MMF. Conclusions Overall, the immunosuppression CTPs were followed as intended in the majority of patients however, adherence to the steroid CTPs was poor indicating revision is necessary. In addition, our pilot study revealed several sources of treatment selection bias that will need to be addressed in for future comparative effectiveness research.

Objectives: This study aims to investigate the growth parameters in Turkish children with systemic lupus erythematosus (SLE) and to compare these growth parameters according to the presence or absence of cyclophosphamide, rituximab treatment, cumulative corticosteroid dose, proliferative nephritis, and the last visit disease activity and damage index. Patients and methods: Medical data, growth parameters including z-scores for weight, height, and body mass index and parent-adjusted height z-scores of 45 juvenile SLE (jSLE) patients (5 males, 40 females; mean age 12.3±3.2 years; range 7.1 to 18 years) were retrospectively evaluated. Growth parameters were calculated by anthropometric references in Turkish children. The disease activity was assessed by the SLE Disease Activity Index 2000 (SLEDAI-2K). The disease outcome was measured by the Pediatric version of Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. Results: The median diagnostic delay was two months (range, 0-36 months). The median follow-up duration was 3.21 years (range, 0.63-9.48 years). Mean height z-score was significantly lower at last visit than at the time of diagnosis. The growth parameters did not differ according to age at disease onset, diagnostic delay, presence of proliferative nephritis, having cyclophosphamide and rituximab treatment and the last visit (SLEDAI-2K) scores. The median duration of corticosteroid treatment was 3.2 years (range, 0.6-9.4 years) and the median cumulative corticosteroid dosage was 13.9 g (range, 1.9-58 g) of methylprednisolone. The mean height z-score at last visit was significantly lower in those who received at least 10 g of cumulative dose of corticosteroid. The last visit mean parent-adjusted height z-scores did not differ significantly regarding the cumulative corticosteroid dose. Conclusion: The last visit height and parent-adjusted height z-scores of jSLE patients were significantly lower. The patients treated with at least 10 g of cumulative dose of corticosteroids had lower mean height z-score. [ABSTRACT FROM AUTHOR]

Aim of the work: To determine the role of Interleukin-34 (IL-34) in the pathogenesis of juvenile systemic lupus erythematosus (J-SLE), by exploring the relationship between IL-34 concentration and the disease activity Subjects and methods: This study was carried out on 48 children with SLE, and 30 healthy control subjects. SLE disease activity was measured by systemic lupus erythematosus disease activity index (SLEDAI). Serum IL-34 was measured by enzyme-linked immunosorbent assay (ELISA). The collected data were statistically analyzed using SPSS program version 16.0. Results: There was a significant elevation in IL-34 concentration in J-SLE patients (52.25 ± 19.94 pg/ml) compared with control group (11.20 ± 6.40 pg/ml) (p < 0.001). The highest level was detected in patients with high SLEDAI score and with lupus nephritis (p = 0.005, 0.003, respectively). There was a statistically significant positive correlation between IL-34 levels and SLEDAI, ESR, CRP, and anti-ds DNA antibodies, but negative correlation with complement (C3, C4), and hemoglobin levels in J-SLE patients. Conclusion: IL-34 could be a probable marker for J-SLE disease activity which is more aggressive than adult-SLE, and IL-34 blockage may suppress the expression of proinflammatory cytokines in patients' blood. Key Points •Juvenile SLE is more aggressive and of worse prognosis than adult-SLE. • Significantly elevated concentration of IL-34 in juvenile SLE patients when compared with controls. • Elevated concentrations of IL-34 in patients are correlated with SLEDAI, ESR, CRP, ds-DNA antibodies, hemoglobin, and complement levels. • IL-34 may play a role in SLE pathogenesis and disease activity. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to determine the prevalence of Fabry disease (FD) among patients with juvenile systemic lupus erythematosus (SLE). Patients and methods: This cross-sectional study included 76 juvenile SLE patients (12 males; 64 females; mean age 16±3.3 years; range, 8 to 23.5 years) who were diagnosed according to 1997 update of the 1982 American College of Rheumatology revised criteria for classification of SLE. Since the majority of patients were female, alpha-galactosidase A gene was investigated for mutations resulting in FD. Lysosomal accumulation of globotriaosylsphingosine (lyso-Gb3) was further evaluated in mutation positive subjects by using dried blood spot testing. Results: Alpha-galactosidase A gene screening did not yield any positive mutation in our 74 subjects. However, a heterozygous p.D313Y mutation was found in two females. These subjects were further investigated for lyso-Gb3 levels in dried blood spot samples and the levels of lyso-Gb3 being normal lead to exclusion of FD in these two patients. Conclusion: We do not suggest routine screening of FD in patients with juvenile SLE; however, prospective studies with larger sample sizes are needed for further analysis. [ABSTRACT FROM AUTHOR]

Introduction/objectives: Tyro3, Axl, and Mer (TAM) receptors and ligands mediate apoptotic bodies engulfment which alteration has been related with juvenile systemic lupus erythematosus (JSLE) pathogenesis. Thus, the aim was to determine their soluble levels. Methods: Serum sTyro3, sAxl, sMer, and Gas6 levels were measured using ELISA in 67 JSLE patients, 12 juvenile idiopathic arthritis (JIA) inflammatory and 20 healthy controls and related with SLEDAI-2K score, anti-dsDNA antibody, ESR, CRP, C3, C4 levels, and nephritis. Results: JSLE patients with active disease (SLEDAI-2K> 4) had significantly increased sMer levels compared with healthy controls (median 8.4 vs. 6.0 ng/mL, p = 0.009) and inactive disease patients (5.2 ng/mL, p = 0.0003). sMer levels correlated with SLEDAI-2K (r = 0.44; p = 0.0004) and ESR (r = 0.24; p = 0.04), while sAxl correlated with SLEDAI-2K (r = 0.33; p = 0.008) and C4 levels (r = − 0.24; p = 0.04). JSLE patients taking glucocorticoid had increased sAxl and sMer levels. Moreover, sAxl correlated with sMer and sTyro3 levels. Patients with nephritis and those with focal or diffuse proliferative glomerulonephritis had these protein levels similar to healthy controls and patients without renal involvement. sTyro3 levels of JSLE patients taking glucocorticoid were decreased, and correlated with Gas6 and sAxl, while Gas6 levels correlated with age upon enrollment. JIA controls had protein levels similar to healthy controls and JSLE patients. Conclusions: This study reinforces that sMer is increased in active JSLE patients, yet sMer and sAxl correlates with disease activity parameters, and their alterations are disease-specific. However, further studies are needed to determine exact roles of sTyro3 and Gas6 in disease pathogenesis. Key Points • sMer and sAxl serum levels are related with active disease in JSLE patients • sMer correlated with SLEDAI-2K score in JSLE • sTyro3, sAxl, sMer and Gas6 levels did not related with nephritis in JSLE patients • sTyro3 and Gas6 exact roles in JSLE are not established and further studies are needed [ABSTRACT FROM AUTHOR]

Purpose: Avascular necrosis (AVN) is a debilitating complication of juvenile systemic lupus erythematosus (jSLE). The aim of this study was to evaluate the frequency and clinical characteristics of patients with AVN in jSLE from a single center. Material and Methods: Fifty-eight jSLE patients diagnosed according to the American College of Rheumatology classification criteria were included in this retrospective study. Disease activity of jSLE patients was measured by SLE Disease Activity Index-2K (SLEDAI- 2K), organ damage was determined by the pediatric version of the systemic lupus international collaborating clinics/American College of Rheumatology damage index (pedSDI) at last visit. Results: Among 58 jSLE patients, the female patients accounted for 86.2% (n=50). Mean baseline SLEDAI-2K score was 21.47±8.96. The number of patients with at least one damage item of pedSDI was 24 (41.4%), in which 5 patients (8.6%) had AVN. There were no statistical differences between the groups except baseline complement 3 (C3) level was significantly lower in patients with AVN. Conclusion: Baseline low C3 level might be a predictor for AVN development in jSLE patients. There is unmet need for multicenter studies investigating possible risk factors of AVN in jSLE patients. [ABSTRACT FROM AUTHOR]

Purpose: Our aim was to evaluate the expression of interleukin-18 (IL-18), interleukin-l-beta (IL-1beta) and the amount of elastase activity in gingival crevicular fluid (GCF) from inflamed gingival sites in patients with juvenile systemic lupus erythematosus (JSLE), and compare these to the expression in GCF from inflamed sites in generally healthy controls. In addition, the local inflammation in periodontal tissues was related to systemic inflammation by the assessment of IL-18 levels in plasma.Materials and Methods: GCF from 16 patients with JSLE and 14 controls were collected using a washing device. Elastase activity was measured with a specific substrate, and IL-18 and 11-1 were measured by ELISA.Results: The percentage of visible plaque index, gingival bleeding index and attachment level were similar in JSLE and controls, while the percentage of probing depth greater or equal to 3 mm was significantly higher in the controls. The total amount of IL-1beta and IL-18 in GCF were significantly decreased in JSLE, while the total amount and the percentage of free elastase activity were significantly higher in JSLE when compared with the controls. The plasma levels of I1-18 and the erythrocyte sedimentation rate were significantly higher in JSLE patients.Conclusion: We found more active elastase in GCF from inflamed sites in JSLE patients even in the presence of significantly lower levels of IL-18 and IL-13. The increased elastase activity suggests a hyperactivity of neutrophils in JSLE, possibly generated by a priming effect caused by the higher plasma levels of IL-18 found in these JSLE patients. [ABSTRACT FROM AUTHOR]