Pediatric Systemic Lupus Erythematosus Research Articles

A Cross-Cultural Validation of a Quality of Life Measure Using the Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY©) among Filipino Pediatric Lupus Patients

Background: Systemic lupus erythematosus (SLE) is one of the most common autoimmune disorders in women of childbearing age. Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) is the only health related quality of life (HRQOL) tool for pediatric SLE, which has been translated into many languages but is not yet available in Filipino. Objective: The primary objective of this study was to develop a Filipino translation of the SMILEY and to test the validity and reliability of this translation. Methodology: The SMILEY was translated into Filipino by a bilingual individual and back-translated by another bilingual individual blinded from the original English version. The translation was evaluated for content validity by a panel of experts and subjected to pilot testing. In the pilot, the SMILEY, together with the previously validated Pediatric Quality of Life Inventory (PEDSQL) 4.0 Generic Core Scale were administered to pediatric lupus patients and their parents on two separate occasions: a baseline and a re-test seven to fourteen days apart. Tests for convergent validity, internal consistency, and test-retest reliability were performed. Results: A total of fifty children and their parents were recruited. The mean age was 15.38±2.62 years (range 8-18 years), mean education level was high school. The mean duration of SLE was 28 months (range 1-81 months). Subjects found the questionnaires to be relevant, easy to understand and to answer. The validity of the SMILEY was demonstrated in terms of content validity, convergent validity, internal consistency, and test-retest reliability. Age, socioeconomic status and educational attainment did not significantly impact the scores. The difference between scores reported by children and parents was significant with SMILEY Total (p=0.0214), effect on Social Life (p=0.0000), and PEDSQL Physical Function (p=0.0460), with children reporting higher scores for these domains compared to their parents. Conclusion: SMILEY is a brief, easy to understand, valid and reliable tool for assessing specific HRQOL in pediatric SLE. It will be useful in providing better care, understanding and may offer critical information regarding the effect of SLE in the quality of life of our pediatric lupus patients. It will help physician understands the needs of their patient not only on treatment of the specific disease but as well as the impact of the treatment on their daily lives.

Introducing a New Dimension to Molecular Disease Classifications

Systemic lupus erythematosus (SLE) is an autoimmune disease presenting with a wide range of clinical manifestations. A recent study in Cell has uncovered distinct molecular classes of pediatric SLE patients. The approach employed is remarkable in that it may enable alignment of molecular classifications of disease with the development and selection of novel treatment modalities.

Recurrent status epilepticus in posterior reversible encephalopathy syndrome as initial feature of pediatric lupus: A newly diagnosed case and literature review

IntroductionPosterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. Systemic lupus erythematosus (SLE), beginning in 15–20% in childhood, is considered as a potential underlying etiology of PRES. In children, status epilepticus (SE) rarely complicates PRES, and exceptionally occurs in SLE. MethodsWe report on an illustrative case of PRES complicating pediatric lupus revealed by recurrent SE, and we further review through a Pubmed search the previously reported cases of pediatric SLE, PRES and SE. ResultsWe describe the case of a 12-year old girl who presented with recurrent status epilepticus associated to high blood pressure and renal involvement. Brain imaging showed classical aspects of PRES. Immunological tests including antinuclear, anti-DNA, and anticardiolipin antibodies were positive. The diagnosis of SLE was established. The Pubmed search identified a total number of 9 children with SE in SLE, and 26 with PRES, including our patient. ConclusionsWe discussed the clinical and paraclinical features of PRES in SLE with epilepsy, their underlying pathophysiological aspects, and their management challenges. PRES should be considered in initial recurrent SE in children, justifying a battery of tests comprising immunological testing. Anticardiolipin antibodies seem to play a crucial role in epilepsy, PRES and renal involvement in pediatric SLE. Further studies are needed to clarify whether PRES should be considered one of the neuropsychiatric manifestations of SLE or a consequence of active disease in other organ systems or its treatment.

Factors associated with thrombosis in pediatric patients with systemic lupus erythematosus.

The risk of thrombosis is increased in patients with systemic lupus erythematosus (SLE). Few studies have assessed factors associated with thrombosis within the pediatric SLE (pSLE) population. We sought to better characterize these associated factors in pSLE patients using the Childhood Arthritis & Rheumatology Research Alliance (CARRA) registry. Within the CARRA registry, patients with a history of thrombosis were compared to those without. Univariate logistic regression was used to calculate odds ratios. A multivariable logistic regression model was conducted that included variables from the univariate analysis that had a p value < 0.10 and other variables identified as clinically significant from published literature. Among the 979 pSLE patients in the CARRA registry, 24 (2.5%) patients had a history of arterial thrombosis and 35 (3.6%) of venous thrombosis. In the univariate analysis, the odds ratio of having a thrombotic event were found to be significantly higher in patients with a history of vasculitis, avascular necrosis (AVN), or antiphospholipid antibody (aPL). Similar results were found for vasculitis, AVN, and aPL in the multivariable analysis. Our study of pSLE patients suggests that vasculitis, positive APL, and AVN are associated with thrombotic events in this population.

Alcohol, smoking and illicit drug use in pediatric systemic lupus erythematosus patients

ObjectiveTo evaluate alcohol, smoking and/or illicit drug use, and history of bullying in adolescent childhood-onset systemic lupus erythematosus and healthy controls. Methods174 adolescents with pediatric rheumatic diseases were selected. All of the 34 childhood-onset systemic lupus erythematosus patients and 35 healthy controls participated in this study. A cross-sectional study included demographic/anthropometric data and puberty markers assessments; structured questionnaire and CRAFFT screening interview. ResultsMcNemar tests indicated an excellent test–retest reliability of the structured questionnaire (p=1.0). The median current age was similar between childhood-onset systemic lupus erythematosus patients and controls [15 (12–18) vs. 15 (12–18) years, p=0.563]. The median of menarche age was significantly higher in childhood-onset systemic lupus erythematosus patients compared to controls [12 (10–15) vs. 11.5 (9–15) years, p=0.041], particularly in those that lupus had occurred before first menstruation [13 (12–15) vs. 11.5(9–15) years, p=0.007]. The other puberty marker and sexual function parameters were similar in both groups (p>0.05). Alcohol use was similar in both childhood-onset systemic lupus erythematosus patients and controls (38% vs. 46%, p=0.628). A trend of lower frequency of CRAFFT score ≥2 (high risk for substance abuse/dependence) was evidenced in childhood-onset systemic lupus erythematosus patients compared to controls (0% vs. 15%, p=0.053). Bullying was reported similarly for the two groups (43% vs. 44%, p=0.950). Further analysis in lupus patients regarding alcohol/smoking/illicit drug use showed no differences in demographic data, puberty markers, history of bullying, sexual function, contraceptive use, disease activity/damage scores, clinical/laboratorial features and treatments (p>0.05). ConclusionThis study showed high frequencies of early alcohol use in lupus adolescents and healthy controls, despite of a possible low risk for substance abuse/dependence in childhood-onset systemic lupus erythematosus patients.

Demographic Presentation, Activity Indices, Damage Index: Comparative Study Between Pediatric Lupus Erythematosus Versus Adult Systemic Lupus Erythematosus in Sample Egyptian Population

Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can affect all organ systems due to alterations of both the innate and adaptive immune systems. Although onset during infancy is rare, the incidence of SLE rises steadily during childhood until mid-adulthood, especially among females. In this study we aimed to highlight the possible discrepancies in clinical presentations as well as serological profiles of pediatric and adult onset SLE patients, we also focused attention on the disease assessment by SLE activity index (SLE DDI) and damage index at time of presentation. Subjects were subdivided into 2 groups: Group I: A total of 92 Pediatric systemic lupus erythematosus (pSLE) that were selected from the students attending the school children hospital of medical health insurance. Group II: A total of 90 adult systemic lupus erythematosus (aSLE) patients and were recruited from those attending the Alexandria Main University Hospital and outpatient clinic. All patients were subjected to: detailed history taking and complete physical and mental examination, also activity indices as well as damage index were applied for every lupus patient of the studied groups, laboratory investigations were done for all patients. Our results demonstrated that, regarding mucocutaneous manifestations: pSLE patients have values higher than aSLE patients regarding photosensitivity (63.3% and 61.1%) and vascular lesions (23.9% and 22.2%) respectively. Regarding haematological manifestations: pSLE patients have values higher than aSLE patients regarding anemia (86.96% and 84.4), leucopenia (28.3% and 22.22) and thrombocytopenia (46.7% and 25.56%) respectively. Regarding renal abnormalities, pSLE patients have higher incidence of nephritic syndrome than aSLE patients. Regarding SLEDAI, pSLE patients have values statistically higher than aSLE patients. Regarding SLAM, pSLE patients have values statistically higher than aSLE patients, while no differences of damage index was noticed.

Nocturnal Hypertension and Attenuated Nocturnal Blood Pressure Dipping is Common in Pediatric Lupus

Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort (n=10), 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus ( p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus ( p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated nocturnal dipping and nocturnal hypertension in SLE patients.

Interleukin-17A rs2275913, Interleukin-17F rs763780 and rs2397084 gene polymorphisms as possible risk factors in Juvenile lupus and lupus related nephritis

There are no reports about the association of interleukin (IL)-17A and IL-17F gene polymorphism and susceptibility to pediatric systemic lupus erythematosus (pSLE). Objective: To examine the possible role of IL-17A rs2275913, IL-17F rs763780 and rs2397084 polymorphisms as risk factors for pSLE in a cohort of Egyptian children and to investigate their association with the clinico-pathological features including lupus nephritis (LN). Methods: Typing of IL-17A and IL-17F polymorphisms was done using restriction fragment length polymorphism for 115 children with SLE and 259 age- and sex-matched healthy controls. Results: No significant differences were found between pSLE patients and healthy controls for the allele and genotype frequencies of IL-17A rs2275913, IL-17F rs763780 and rs2397084 (p > 0.05). However, the combined genotype GGAGAA and the haplotype GGA had significant association with pSLE (pc = 0.042 and <0.001, respectively). The AA genotype of IL-17F rs763780 is more frequent in female patients (p = 0.002) and the AA genotype of IL-17F rs2397084 is more associated with positivity of ds-DNA (p = 0.007). No more associations were found for the demographic and clinical data of pSLE patients including risk of LN development, risk of non-remission, overall survival, activity and chronicity indices. Conclusion: The GGAGAA combined genotype and the GGA haplotype of IL-17A rs2275913, IL-17F rs763780 and rs2397084 can be considered risk factors for the development of SLE in Egyptian children. IL-17A rs2275913, IL-17F rs763780 and rs2397084 are not related to the LN development, SLE disease activity or overall survival.

Nocturnal Hypertension and Attenuated Nocturnal Blood Pressure Dipping is Common in Pediatric Lupus

Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort (n=10), 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus ( p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus ( p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated nocturnal dipping and nocturnal hypertension in SLE patients.

E coli septic arthritis of the knee: A rare complication in pediatric lupus

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

Nocturnal Hypertension and Attenuated Nocturnal Blood Pressure Dipping is Common in Pediatric Lupus

Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort, 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus (p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus (p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated nocturnal dipping and nocturnal hypertension in SLE patients.

Practice patterns and approach to kidney biopsy in lupus: a collaboration of the Midwest Pediatric Nephrology Consortium and the Childhood Arthritis and Rheumatology Research Alliance.

BackgroundThere is no clear consensus regarding optimal indications or timing of initial or repeat kidney biopsy in the management of pediatric systemic lupus erythematosus (pSLE).MethodsA web-based survey was designed to assess current practice patterns among pediatric nephrologists and pediatric rheumatologists and distributed to members of Midwest Pediatric Nephrology Consortium (MWPNC) and Childhood Arthritis and Rheumatology Research Alliance (CARRA).ResultsRespondents included 111 rheumatologists and 71 nephrologists from 65 and 34 centers, respectively. Numbers of years in sub-specialty practice were comparable. Rheumatologists and nephrologists frequently collaborate in the care of children with lupus nephritis (LN). More than 90 % of respondents refer patients to each either other after diagnosing LN. Over 60 % describe shared decision making regarding when to perform kidney biopsy and how to interpret biopsy findings. Many pediatric nephrologists consider biopsy to be of higher risk for complication in pSLE and alter their standard pre-or post-biopsy management.ConclusionsIt is uncommon for pediatric nephrologists to manage LN without input from pediatric rheumatologists and vice versa. Consensus exists between specialties in general, and practice differences that exist occur between individual physicians rather than between specialties. A systematic approach to biopsy may result in improved health related outcomes in pSLE.Electronic supplementary materialThe online version of this article (doi:10.1186/s12969-015-0024-x) contains supplementary material, which is available to authorized users.

Autoantigen microarrays reveal autoantibodies associated with proliferative nephritis and active disease in pediatric systemic lupus erythematosus.

IntroductionPediatric systemic lupus erythematosus (pSLE) patients often initially present with more active and severe disease than adults, including a higher frequency of lupus nephritis. Specific autoantibodies, including anti-C1q, anti-DNA and anti-alpha-actinin, have been associated with kidney involvement in SLE, and DNA antibodies are capable of initiating early-stage lupus nephritis in severe combined immunodeficiency (SCID) mice. Over 100 different autoantibodies have been described in SLE patients, highlighting the need for comprehensive autoantibody profiling. Knowledge of the antibodies associated with pSLE and proliferative nephritis will increase the understanding of SLE pathogenesis, and may aid in monitoring patients for renal flare.MethodsWe used autoantigen microarrays composed of 140 recombinant or purified antigens to compare the serum autoantibody profiles of new-onset pSLE patients (n = 45) to healthy controls (n = 17). We also compared pSLE patients with biopsy-confirmed class III or IV proliferative nephritis (n = 23) and without significant renal involvement (n = 18). We performed ELISA with selected autoantigens to validate the microarray findings. We created a multiple logistic regression model, based on the ELISA and clinical information, to predict whether a patient had proliferative nephritis, and used a validation cohort (n = 23) and longitudinal samples (88 patient visits) to test its accuracy.ResultsFifty autoantibodies were at significantly higher levels in the sera of pSLE patients compared to healthy controls, including anti-B cell-activating factor (BAFF). High levels of anti-BAFF were associated with active disease. Thirteen serum autoantibodies were present at significantly higher levels in pSLE patients with proliferative nephritis than those without, and we confirmed five autoantigens (dsDNA, C1q, collagens IV and X and aggrecan) by ELISA. Our model, based on ELISA measurements and clinical variables, correctly identified patients with proliferative nephritis with 91 % accuracy.ConclusionsAutoantigen microarrays are an ideal platform for identifying autoantibodies associated with both pSLE and specific clinical manifestations of pSLE. Using multiple regression analysis to integrate autoantibody and clinical data permits accurate prediction of clinical manifestations with complex etiologies in pSLE.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0682-6) contains supplementary material, which is available to authorized users.

OX40 Ligand Contributes to Human Lupus Pathogenesis by Promoting T Follicular Helper Response

Increased activity of T follicular helper (Tfh) cells plays a major pathogenic role in systemic lupus erythematosus (SLE). However, the mechanisms that cause aberrant Tfh cell responses in SLE remain elusive. Here we showed the OX40 ligand (OX40L)-OX40 axis contributes to the aberrant Tfh response in SLE. OX40L was expressed by myeloid antigen-presenting cells (APCs), but not B cells, in blood and in inflamed tissues in adult and pediatric SLE patients. The frequency of circulating OX40L-expressing myeloid APCs positively correlated with disease activity and the frequency of ICOS(+) blood Tfh cells in SLE. OX40 signals promoted naive and memory CD4(+) Tcells to express multiple Tfh cell molecules and were sufficient to induce them to become functional B cell helpers. Immune complexes containing RNA induced OX40L expression on myeloid APCs via TLR7 activation. Our study provides a rationale to target the OX40L-OX40 axis as a therapeutic modality for SLE.

Apartheid and healthcare access for paediatric systemic lupus erythematosus patients in South Africa

South Africa (SA) still faces the legacy of apartheid: the history, politics and economics have a lasting, indelible effect on the health of its people. Here, we discuss the challenges of caring for patients with chronic disease, focusing on paediatric systemic lupus erythematosus (SLE), as a framework for evaluating the structural challenges to accessing care in SA. Prior reports have demonstrated numerous factors influencing access for SA patients with chronic disease. This article explores the economic, social and political vestiges of apartheid as it affects the healthcare system and subsequently, our patients.

Incidence and prognosis of systemic lupus erythematosus in a 35 year period in Fukushima, Japan.

We examined the epidemiology, clinical manifestations, and prognosis of pediatric systemic lupus erythematosus (SLE) in Fukushima Prefecture, Japan over a 35 year period. We collected the medical records of 37 patients diagnosed with SLE between 1977 and 2013. These children were divided into two groups. group 1 consisted of 19 patients who were diagnosed between 1977 and 1995, and group 2 consisted of 18 patients diagnosed between 1996 and 2013. The epidemiology, clinical features, and prognosis were retrospectively compared between the two groups. The mean number of patients per 100,000 children per year for group 1 and group 2 was 0.33 ± 0.25 and 0.35 ± 0.30, respectively. The duration from onset of symptoms to treatment in group 2 was shorter than that in group 1, but the clinical and laboratory findings at onset did not differ between the two groups. All patients were treated with prednisolone, and 17 patients in group 1 and 18 in group 2 were treated with methylprednisolone pulse therapy. The frequency of cyclophosphamide treatment decreased whereas the frequency of cyclosporine, tacrolimus and mizoribine pulse therapy increased in group 2. SLE disease activity index (SLEDAI) score at the latest follow up in group 2 was lower in group 1. The survival rate was 84% in group 1 and 100% in group 2. The frequency and severity of SLE in group 1 were similar to those in group 2, and the prognosis of SLE in group 2 was better than that in group 1.

High Titer Anti-Basement Membrane Antibodies in a Subset of Patients with Pediatric Systemic Lupus Erythematosus

Background/Aims: There is a critical need for more noninvasive biomarkers to identify nephritis in patients with systemic lupus erythematosus (SLE). Recent studies in a model mouse and an adult SLE patient cohort suggest that anti-basement membrane antibody levels correlate well with lupus activity and kidney injury. The purpose of this study was to assess the anti-basement membrane reactivity in pediatric SLE (pSLE) patients with or without nephritis. Methods: Auto-antibodies to basement membrane antigens were assessed using an anti-matrigel ELISA. Endpoint titers were measured in pSLE patients and healthy children, as well as in autoimmune and non-immune mice, with good reproducing capabilities. Findings were also analyzed with respect to the presence or absence of nephritis, dsDNA antibodies, and other manifestations of pSLE. Results: MRL/lpr mice developed high-titer anti-matrigel antibodies, whereas C57BL/6 mice did not. In a cohort of 21 pSLE patients and 22 pediatric controls, high-titer anti-matrigel IgG, IgM and IgA antibody levels were specific for pSLE. High-titer anti-matrigel IgG3 levels could distinguish with good sensitivity the 13 pSLE patients with a history of nephritis from the 8 non-renal pSLE patients. High-titer anti-matrigel IgG, IgA, IgM or IgG3 did not correlate with positive anti-double stranded DNA, but defined an overlapping subset of patients. Conclusion: The addition of anti-basement membrane antibody testing to serologic testing in pSLE may help to monitor disease activity or to define important subsets of patients with risks for specific disease manifestations.

Avascular necrosis in pediatric systemic lupus erythematosus: a brief report and review of the literature.

ᅟAvascular necrosis (AVN) occurs in several chronic illnesses, including systemic lupus erythematosus (SLE), but can also occur in healthy children. There are multiple theories to explain why and how AVN occurs, but an exact mechanism has yet to be unraveled. AVN in the pediatric lupus population is understudied. The Atherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) trial, provides an excellent venue to conduct an exploratory analysis to assess associations between AVN and demographics, SLE disease activity and vitamin D deficiency. Herein we present a brief report describing our findings, as well as reviewing the literature on AVN in SLE and other entities.Trial registrationClinicalTrials.gov identifier: NCT00065806.

Pediatric systemic lupus erythematosus presenting with coronary arteritis: A case series and review of the literature

ObjectivePediatric-onset systemic lupus erythematosus (pSLE) is typically more aggressive in presentation than adult-onset lupus. Presenting manifestations of lupus in children and adults involve similar organ systems, with renal and neuropsychiatric involvement more common in pSLE. Cardiac manifestations are similar in the 2 groups, with pericarditis accounting for the majority of cardiac lupus at presentation. There are no reports to our knowledge of coronary arteritis as a presenting feature of pSLE. MethodsThis is a retrospective case series describing 4 pediatric lupus patients who presented with prominent coronary artery dilatation and a review of the literature regarding coronary artery involvement in lupus. ResultsCoronary arteritis appears to be a more common feature of pSLE than previously thought. Based on our experience, coronary artery changes tend to resolve once the SLE is treated. ConclusionsEarly recognition of this disease manifestation may guide therapy and result in improved long-term cardiovascular outcomes.

Clinical Presentation of Pediatric SLE at Outpatient Clinic

Background: Systemic lupus erythematosus (SLE) is a rheumatic disease resulting in damage to any organ in the body.10-20% of SLE patients diagnosed in childhood. Objective: To define the pattern of disease expression in pediatric SLE. Sitting: Outpatient clinic of pediatric SLE of Abu Alrich hospital-Cairo-Egypt. Subject: 25 pediatric SLE patients who attend the clinic. Design of study: observational descriptive case series report. Data collection: Data collected by second author during her residency in Abu Alrich hospital to study rheumatology. The 25 patients randomly selected. She collects medical history information directly from the patients and their parents and reviews their files to collect the other information (e.g.: investigation results and clinical examination and finding during course of the disease). Results: From 25 patients Female to male ratio 2.3:1. Age at diagnosis range from 4 to 15 years with mean of 9 years. Duration of symptoms before diagnosis established range from one month to 7 years with mean of 8 months. Frequency of SLE classification criteria as follows: +ve ANA 95%, renal disorder: 84%, immunological disorder 60%, photosensitivity 48%, oral ulcers 48%, Hematological disorder 44%, arthritis 36%, serositis 28%, malar rash 12%, discoid rash 4%, neurological disorder 0%. Other clinical manifestation of SLE include fever 80%, arthralgia and anorexia 84% each, hair fall 40%, abdominal pain 36%, other skin manifestation 24%, headache 20%,dyspnea and palpitation 16% each, Reynard’s phenomena and lymphadenopathy 12% each. The most frequent hematological abnormality: elevated ESR: 100% anemia: 72%, leucopenia: 44%, thrombocytopenia: 20%, leukocytosis: 12%, thrombocytosis 8%, lymphopenia: 4%. Conclusion and recommendation: The presentation and the clinical course of SLE have wide variability. Nephropathy, fever, anorexia and arthralgia were the most common presenting clinical manifestations in pediatric patients. There is marked delay between the starting of the disease manifestation and diagnosis, so alertness to the possibility of SLE as diagnosis in patients with variable manifestation should