Juvenile Systemic Lupus Erythematous Research Articles

Demographic data of Egyptian juvenile systemic lupus erythematosus during COVID-19 pandemic

Background: Juvenile Systemic lupus erythematous is a chronic autoimmune inflammatory disease that causes damage to various organs such as kidneys, blood, musculoskeletal and nervous system. SLE is variable ranging From mild to severe with unpredictable course of the disease and periods of remission and flares. Methods : The study included 67 patients diagnosed as having jSLE according to the Systemic Lupus International Collaborating Clinics(SLICC) for whom demographic data , full history and laboratory investigations were collected. Results: Among our study population were 13.4%males and 86.6% females with male to female ratio (1:6.4 ). Their mean age was 10.94±3 years. Most of our patients were from Giza (43.3%) followed by Cairo (22.4%). C3 and C4 were consumed in 74.6% of patients, proteinuria was present in 73.1% of patients , hematuria was present in 25.4% of patients. ANA was positive in 98.5% of patients at the time of diagnosis while antiDNA was positive in 85.1%. Conclusion: Juvenile systemic lupus erythematosus is not uncommon in Egypt with female predominance as known worldwide but younger age of onset of the disease. During COVID 19 pandemic follow up was limited due to fear of infection.

Tocilizumab for massive refractory pleural effusion in an adolescent with systemic lupus erythematosus

BackgroundPleural effusion in systemic lupus erythematous (SLE) is a common symptom, and recent studies demonstrated that IL-6 has a pivotal role in its pathogenesis.Case presentationWe report a case of a 15 years old Caucasian boy with a history of persistent pleural effusion without lung involvement or fever. Microbiological and neoplastic aetiologies were previously excluded. Based on the presence of pleuritis, malar rash, reduction of C3 and C4 levels and positivity of antinuclear antibody (ANA) and anti-double stranded DNA (dsDNA), the diagnosis of juvenile SLE (JSLE) was performed. Treatment with high dose of intravenous glucocorticoids and mycophenolate mofetil was started with partial improvement of pleural effusion. Based on this and on adults SLE cases with serositis previously reported, therapy with intravenous tocilizumab (800 mg every two weeks) was started with prompt recovery of pleural effusion.ConclusionTo the best of our knowledge, this is the first case of JSLE pleuritis successfully treated with tocilizumab.

Pseudotumor Cerebri as the First Manifestation of Juvenile Systemic Lupus Erythematosus

IntroductionHeadache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed intractable headache that was eventually diagnosed as PTC and presented as the first manifestation of JSLE.Case PresentationA 9-year-old girl with a history of progressive headache for four months, fever, fatigue, myalgia, arthralgia, small-joint arthritis of the hands, and recent diplopia was referred to our clinic. The diagnosis of PTC in the background of JSLE was made based on her history, physical examination, positive laboratory findings, and increased intracranial pressure, with normal neuroimaging. Treatment with high-dose prednisolone led to dramatic resolution of the headache.ConclusionsIt seems that a complete neurologic examination in newly diagnosed SLE patients is mandatory, especially in the presence of any neuropsychiatric manifestations, such as headache.

Role of microRNA-22 and microRNA-1825 in the diagnosis and differential diagnosis of juvenile systemic lupus erythematous

Objective To explore the role of microRNA(miR)-22 and miR-1825 in the diagnosis and differential diagnosis of juvenile systemic lupus erythematous(JSLE). Methods The cases of JSLE hospitalized in Capital Institute of Pediatrics Teaching Hospital Affiliated to Peking University from June 2013 to May 2014 were selected as study group.The cases with systemic juvenile idiopathic arthritis(sJIA), nephrotic syndrome(NS), Kawasaki disease(KD), Henoch-Schonlein purpura(HSP)were selected as patients control group.The healthy children were selected as healthy control group.The expression levels of miR-22 and miR-1825 in the plasma of JSLE, sJIA, NS, KD, HSP and healthy children were detected by using real-time PCR respectively.Receiver operating characteristic curve (ROC) analysis was performed to evaluate the value of miR-22 and miR-1825 miRNA as a biomarker with the sensitivity and specificity.Three data bases, included Targetscan, PicTar and miRanda, were applied to predict the target gene.The target gene was analyzed by adopting Gene Ontology (GO) in terms of molecular function, biological process and cellular component, and by adopting Kyoto Encyclopedia of Genes and Genomes(KEGG) in terms of pathway. Results Compared with healthy children, the amount of miR-22 and miR-1825 in JSLE patients were lower, and there were significant differences(t=-3.076, -9.054, P 0.05). The area under ROC curve(AUC) of miR-22 between JSLE and healthy children was 0.777.The AUC of miR-1825 between JSLE and healthy children was 1.000.The AUCs between JSLE and controls of sJIA, NS, KD, HSP of miR-22 were 0.731-1.000.The AUCs between JSLE and controls of sJIA, NS, KD, HSP of miR-1825 were 0.939-1.000.There was positive relation between the amount of miR-22 and complement C3 in plasma(r=0.493, P=0.027). Conclusions The amount of miR-22 and miR-1825 in the plasma of JSLE embrace the potential of distinguishing JSLE from healthy children, sJIA, NS, KD, HSP.MiR-22 has the ability to predict the activity of JSLE. Key words: Juvenile systemic lupus erythematous; MicroRNA-22; MicroRNA-1825; Diagnosis; Differential diagnosis

Juvenile systemic lupus erythematous in Iranian children

Results A total of 59 patients, 49 (89%) were female and10 (16.9%) male. The female to male ratio was 4.9:1. The mean age of onset was 10.5 years (range 2-16). The mean duration of disease from diagnosis was 2 years (range7 months-5 years). The most common of constitutional sign was fatigue (76.3%) and the other signs were fever (32.2%), Nocturnal pain (20.3%), Mood disorder (20.03%), and Weight loss (22%). The most common manifestation of JSLE were as follows: 48 patients (81.35%) had mucocutaneous involvement, 46 patients (77.9%) had musculoskeletal involvement, 25 children (42.37%) suffered from renal disease, hematological abnormalities were detected in 19 patients (32.2%), 10 patients (16.94%) had cardiovascular disease, 33 patients (55.93%) presented nervous system involvement, 12 patients (20.33%) had pulmonary disease, and 13 patients (22.03) experienced infection complications. During the follow up period(3years) two patients died, one from renal failure, one from infections. Conclusion A high index of suspicion must be preserved for the diagnosis of SLE in adolescent children, particularly girls. Juvenile systemic lupus erythematous (JSLE) is a critical multi-system organ disease. JSLE still has a significant mortality rate high, although it has a high remission rate with early diagnosis and treatment.

PReS-FINAL-2291: Activation of TLR pathway JSLE derived neutrophil extracellular traps

Juvenile Systemic Lupus Erythematous (JSLE) is characterised by auto-antibody production directed against nuclear auto-antigens. Toll-like receptors (TLRs) are pattern recognition receptors of the innate immune system responsible for initiating an immune response against invading pathogens. TLR 3, 7-9 have been studied in SLE due to their unique ability to detect nuclear antigen. Their expression is increased in JSLE and significantly associated with disease activity and anti-dsDNA titres. Upon recognition of an extracellular pathogen, neutrophils may release neutrophil extracellular traps (NETs) containing antimicrobial peptides to capture and neutralise pathogens. Nuclear material including DNA and histones comprise the major structural components of NETs and may act as a source of nuclear auto-antigen in SLE. The mechanism by which NETs may induce an auto-inflammatory response has not been elucidated. Our hypothesis is that neutrophil NETs are a source of nuclear auto-antigen in JSLE being detected through the TLR pathway leading to an auto-inflammatory response.

PReS-FINAL-2286: Mastitis in an adolescent patient with juvenile systemic lupus erythematous

Mastitis is an inflammatory disease of the breast, acute or chronic that occurs primarily in young women and frequently lactating. In 2-3% it may occur in patients with systemic lupus erythematous (SLE). The authors describe in an adolescent with JSLE this rare disease called lupus mastitis, which is a subset of lupus panniculitis limited to the breast.

PReS-FINAL-2287: Electroconvulsive therapy in a patient with juvenile systemic lupus erythematous

Patients with systemic lupus erythematous (SLE) often develop neuropsychiatric disease. Central nervous system (CNS) manifestations of SLE occur in about half of all patients, and psychiatric presentations are seen with a prevalence of 35% to 60% in this group. Electroconvulsive therapy (ECT) also known as electroshock therapy is a psychiatric treatment in which electricity/electric shock is used to induce convulsions. It is used to treat psychiatric diseases such as depression, schizophrenia, mania or catatonia that do not respond to conventional treatment.

Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases

Macrophage activation syndrome (MAS) belongs to secondary hemophagocytic lymphohistiocytosis (HLH) syndrome. It is usually associated with rheumatic diseases. We retrospectively reviewed our hospital's medical records of 102 HLH/MAS patients from the past 20 years. Demographics, clinical data, treatment, and outcomes were analyzed. Among 102 patients, eight patients with underlying juvenile systemic lupus erythematous (two patients), mixed connective tissue disease (one patient), primary anti-phospholipid syndrome (one patient), and systemic type juvenile rheumatoid arthritis (sJRA; four patients) with 13 episodes of MAS were studied. Clinical manifestations of MAS included fever (100 %), hepatosplenomegaly (77 %), lymphadenopathy (38 %), skin rash (62 %), and neurological involvement (31 %). Laboratory features included leukopenia (54 %), anemia (46 %), thrombocytopenia (77 %), jaundice (27 %), hypofibrinogenemia (40 %), decreased erythrocyte sedimentation rate (67 %), and elevated liver enzymes (77 %), lactate dehydrogenase (100 %), ferritin (88 %), triglycerides (91 %), C-reactive protein (85 %), plasma D-dimer (50 %), and hemophagocytosis in bone marrow (83 %). The Epstein-Barr virus and adenovirus infection triggered MAS in two patients with sJRA. Methylprednisolone pulse therapy was effective in two out of three patients, and high-dose intravenous immunoglobulin (IVIG) was effective in two out of six patients. Patients with sJRA responded well to corticosteroids and cyclosporine. Complications included opportunistic infection with Pneumocystis jiroveci, multiple organ failure, and intensive care unit myopathy. The mortality rate was one out of eight (12.5 %). Our results showed that MAS could be fatal and complicate various pediatric autoimmune diseases. It generally has a good response to corticosteroids and IVIG. Prompt recognition and timely treatment can result in good outcomes.

Juvenile systemic lupus erythematosus: Review of clinical features and management

879 VOLUME 48__NOVEMBER 17, 2011 Juvenile systemic lupus erythematous (SLE) is a multisystem autoimmune disorder that is characterized by widespread immune dysregulation, formation of autoantibodies and immune complexes, resulting in inflammation and potential damage to a variety of organs. It is not uncommon for children to present with non-specific symptoms, and little else, and be treated for a presumed infection, including tuberculosis – with subsequent evaluation revealing the diagnosis of lupus, requiring aggressive management. A high index of suspicion must be maintained for the diagnosis of SLE in adolescent children, particularly girls.

Use of Intravenous Immunoglobulin Therapy in a paediatric rheumatology service

Background Intravenous Immunoglobulin (IVIG) is widely used in paediatric inflammatory disease. The Department of Health, UK recently produced guidelines for IVIG use and a demand management plan in response to global IVIG shortages, recommending use in selected patients with juvenile dermatomyositis (JDMS), as a last resort in systemic juvenile idiopathic arthritis (sJIA), and use not supported in juvenile systemic lupus erythematous (jSLE) and vasculitis.