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https://www.readbyqxmd.com/read/27771618/juvenile-systemic-lupus-erythematosus-in-nigeria
#1
O O Adelowo, B H Olaosebikan, B A Animashaun, R O Akintayo
Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014...
October 21, 2016: Lupus
https://www.readbyqxmd.com/read/27590021/international-validation-of-a-urinary-biomarker-panel-for-identification-of-active-lupus-nephritis-in-children
#2
Eve Mary Dorothy Smith, Andrea Lyn Jorgensen, Angela Midgley, Louise Oni, Beatrice Goilav, Chaim Putterman, Dawn Wahezi, Tamar Rubinstein, Diana Ekdawy, Rachel Corkhill, Caroline Ann Jones, Stephen David Marks, Paul Newland, Clarissa Pilkington, Kjell Tullus, Michael William Beresford
BACKGROUND: Conventional markers of juvenile-onset systemic lupus erythematosus (JSLE) disease activity fail to adequately identify lupus nephritis (LN). While individual novel urine biomarkers are good at detecting LN flares, biomarker panels may improve diagnostic accuracy. The aim of this study was to assess the performance of a biomarker panel to identify active LN in two international JSLE cohorts. METHODS: Novel urinary biomarkers, namely vascular cell adhesion molecule-1 (VCAM-1), monocyte chemoattractant protein 1 (MCP-1), lipocalin-like prostaglandin D synthase (LPGDS), transferrin (TF), ceruloplasmin, alpha-1-acid glycoprotein (AGP) and neutrophil gelatinase-associated lipocalin (NGAL), were quantified in a cross-sectional study that included participants of the UK JSLE Cohort Study (Cohort 1) and validated within the Einstein Lupus Cohort (Cohort 2)...
September 3, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27502765/contraception-for-adolescents-with-chronic-rheumatic-diseases
#3
Benito Lourenço, Katia T Kozu, Gabriela N Leal, Marco F Silva, Elisabeth G C Fernandes, Camila M P França, Fernando H C Souza, Clovis A Silva
Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies...
July 16, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27373868/urinary-biomarkers-in-childhood-lupus-nephritis
#4
Eve M D Smith, Michael W Beresford
Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, severe multisystem autoimmune disease affecting the kidney (Lupus Nephritis, LN) in up to 80% of children. LN is more severe in children than adults, with potential for irreversible kidney damage requiring dialysis or transplant. Renal biopsy is currently the gold standard for diagnosing and monitoring LN, however, it is invasive and associated with complications. Urine biomarkers have been shown to be better than serum biomarkers in differentiating renal disease from other organ manifestations...
June 29, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27267647/miliary-tuberculosis-a-severe-opportunistic-infection-in-juvenile-systemic-lupus-erythematosus-patients
#5
Priscilla S Freire, João D Montoni, Aline S M Ribeiro, Heloísa H Marques, Thais Mauad, Clovis A Silva
INTRODUCTION: One of the main issues in juvenile systemic lupus erythematosus (JSLE) patients is infection, such as tuberculosis (TB). Of note, SLE patients are susceptible to pulmonary and extrapulmonary TB. However, to our knowledge, this contagious disease was rarely reported in pediatric lupus population, particularly diffuse or miliary TB. Therefore, from January 1983 to December 2011, 5,635 patients were followed-up at our Pediatric Rheumatology Unit and 285 (5%) of them met the American College of Rheumatology classification criteria for SLE...
May 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27267644/association-between-academic-performance-and-cognitive-dysfunction-in-patients-with-juvenile-systemic-lupus-erythematosus
#6
Renan Bazuco Frittoli, Karina de Oliveira Peliçari, Bruna Siqueira Bellini, Roberto Marini, Paula Teixeira Fernandes, Simone Appenzeller
OBJECTIVE: To determine whether there is an association between the profile of cognitive dysfunction and academic outcomes in patients with juvenile systemic lupus erythematosus (JSLE). METHODS: Patients aged ≤18 years at the onset of the disease and education level at or above the fifth grade of elementary school were selected. Cognitive evaluation was performed according to the American College of Rheumatology (ACR) recommendations. Symptoms of anxiety and depression were assessed by Beck scales; disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); and cumulative damage was assessed by Systemic Lupus International Collaborating Clinics (SLICC)...
May 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27267642/hepatitis-c-virus-antibodies-in-high-risk-juvenile-onset-systemic-lupus-erythematosus
#7
Nádia E Aikawa, Ana P Nascimento, André L S Hayata, Eloisa Bonfá, Cláudia Goldenstein-Schainberg
OBJECTIVE: To evaluate the prevalence of hepatitis C virus (HCV) infection in high risk juvenile systemic lupus erythematosus (JSLE). STUDY DESIGN: Forty low income JSLE patients (6M:34F; mean age 19±4.4 yrs; mean disease duration 6±3.2 yrs) were studied. Twenty healthy children and adolescents matched for social economical level were included as controls. Anti-HCV tests were performed using a third generation microparticle enzyme immunoassay. Inclusion criterion was low social economical level...
May 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27255473/association-of-interleukin-2-and-interferon-%C3%AE-single-nucleotide-polymorphisms-with-juvenile-systemic-lupus-erythematosus
#8
S Harsini, V Ziaee, F Tahghighi, M Mahmoudi, A Rezaei, S Soltani, M Sadr, M H Moradinejad, Y Aghighi, N Rezaei
PURPOSE: Juvenile systemic lupus erythematosus (JSLE) is a severe and chronic autoimmune disease of unknown origin. Inflammatory cytokines can play a pivotal role in the pathogenesis of JSLE, while their secretion is under genetic control. The current investigation was performed to analyse the associations of particular single nucleotide polymorphisms (SNPs) of interleukin-2 (IL-2) and interferon-gamma (IFN-γ) genes in a case control study. MATERIALS AND METHODS: The allele, genotype and haplotype frequencies of the polymorphic IL-2 (G/T at -330, rs2069762, and G/T at +166, rs2069763) and IFN-γ (A/T at +874, rs2430561) genes were estimated in 59 patients with JSLE by contrast with 140 healthy controls using polymerase chain reaction with sequence-specific primers method...
September 2016: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/27230556/correlation-between-the-modified-systemic-lupus-erythematosus-disease-activity-index-2000-and-the-european-consensus-lupus-activity-measurement-in-juvenile-systemic-lupus-erythematosus
#9
J O Sato, J E Corrente, C Saad-Magalhães
OBJECTIVE: The objective of this study was to assess Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and European Consensus Lupus Activity Measurement (ECLAM) disease activity correlation in addition to their respective correlation to Pediatric Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (Ped-SDI), in juvenile systemic lupus erythematosus (JSLE). METHODS: The activity indices were scored retrospectively and summarized by adjusted means during follow-up...
May 26, 2016: Lupus
https://www.readbyqxmd.com/read/27147622/differences-in-disease-phenotype-and-severity-in-sle-across-age-groups
#10
N Ambrose, T A Morgan, J Galloway, Y Ionnoau, M W Beresford, D A Isenberg
OBJECTIVES: Significant differences have been reported in disease phenotype and severity of systemic lupus erythematosus (SLE) presenting in different age groups. Most indicate a more severe phenotype in juvenile-onset SLE (JSLE). There have been limited studies in older patients and no large studies looking at SLE across all age groups. METHODS: We assessed the effect of age of onset of SLE on the clinical phenotype by analysing data from two large UK cohorts (the UK JSLE Cohort and the UCLH SLE cohort)...
December 2016: Lupus
https://www.readbyqxmd.com/read/27115107/anti-cd20-rituximab-therapy-in-refractory-pediatric-rheumatic-diseases
#11
J Reis, F Aguiar, I Brito
OBJECTIVES: We aim to report the efficacy and safety of rituximab (RTX) in patients diagnosed with juvenile systemic lupus erythematosus (JSLE) or juvenile idiopathic arthritis (JIA) refractory to conventional treatment. METHODS: A retrospective review was made of all medical records of patients with JSLE or JIA treated with RTX between January 2009 and January 2015 in the Pediatric Rheumatology Unit of a central hospital. RESULTS: Five patients, 4 with JSLE and 1 with extended oligoarticular JIA, received 10 cycles of RTX (23 infusions)...
January 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/27021334/poor-agreement-of-objectively-measured-and-self-reported-physical-activity-in-juvenile-dermatomyositis-and-juvenile-systemic-lupus-erythematosus
#12
Ana Jéssica Pinto, Hamilton Roschel, Fabiana Braga Benatti, Ana Lúcia de Sá Pinto, Adriana Maluf Elias Sallum, Clóvis Arthur Silva, Bruno Gualano
To examine the agreement and association between objectively measured and indirectly assessed physical activity levels in patients with juvenile dermatomyositis (JDM) and juvenile systemic lupus erythematosus (JSLE) patients. The sample consisted of 19 JDM patients (age 8 to 22 years) and 20 JSLE patients (age 9 to 18 years). Physical activity level was objectively measured using Actigraph® accelerometers and indirectly assessed by the short-form International Physical Activity Questionnaire (IPAQ). Spearman's correlation coefficients were calculated to test possible associations between physical activity levels across the two instruments...
June 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27012518/association-between-academic-performance-and-cognitive-dysfunction-in-patients-with-juvenile-systemic-lupus-erythematosus
#13
Renan Bazuco Frittoli, Karina de Oliveira Peliçari, Bruna Siqueira Bellini, Roberto Marini, Paula Teixeira Fernandes, Simone Appenzeller
OBJECTIVE: To determine whether there is an association between the profile of cognitive dysfunction and academic outcomes in patients with juvenile systemic lupus erythematosus (JSLE). METHODS: Patients aged ≤18 years at the onset of the disease and education level at or above the fifth grade of elementary school were selected. Cognitive evaluation was performed according to the American College of Rheumatology (ACR) recommendations. Symptoms of anxiety and depression were assessed by Beck scales; disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); and cumulative damage was assessed by Systemic Lupus International Collaborating Clinics (SLICC)...
March 8, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/26949148/hepatitis-c-virus-antibodies-in-high-risk-juvenile-onset-systemic-lupus-erythematosus
#14
Nádia E Aikawa, Ana P Nascimento, André L S Hayata, Eloisa Bonfá, Cláudia Goldenstein-Schainberg
OBJECTIVE: To evaluate the prevalence of hepatitis C virus (HCV) infection in high risk juvenile systemic lupus erythematosus (JSLE). STUDY DESIGN: Forty low income JSLE patients (6M:34F; mean age 19±4.4 yrs; mean disease duration 6±3.2 yrs) were studied. Twenty healthy children and adolescents matched for social economical level were included as controls. Anti-HCV tests were performed using a third generation microparticle enzyme immunoassay. Inclusion criterion was low social economical level...
February 17, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/26854079/increased-concentration-of-plasma-tnfr1-and-tnfr2-in-paediatric-lupus-nephritis
#15
M Patel, L Oni, A Midgley, E Smith, K Tullus, S D Marks, C A Jones, C Pilkington, M W Beresford
BACKGROUND: Juvenile-onset systemic lupus erythematous (JSLE) is a debilitating condition that frequently involves the kidneys (lupus nephritis; LN). Tumour necrosis factor alpha (TNF-α), an important pro-inflammatory cytokine, is expressed locally in the kidney and correlates with LN disease activity. The aim of this study was to ascertain whether soluble receptors for TNF-α (sTNFR1/sTNFR2) are significantly increased in children with LN. METHODS: Plasma samples were collected from JSLE patients at routine review...
August 2016: Lupus
https://www.readbyqxmd.com/read/26800705/low-c4-c4a-and-c4b-gene-copy-numbers-are-stronger-risk-factors-for-juvenile-onset-than-for-adult-onset-systemic-lupus-erythematosus
#16
MULTICENTER STUDY
Kaline M C Pereira, Atila G A Faria, Bernadete L Liphaus, Adriana A Jesus, Clovis A Silva, Magda Carneiro-Sampaio, Luis E C Andrade
OBJECTIVE: Complete deficiency of Complement C4 component is a strong genetic risk factor for SLE. C4 is encoded by two different genes, C4A and C4B, which show considerable gene copy number (GCN) variation. This study investigates the association of total C4, C4A and C4B GCN with JSLE. METHODS: Ninety JSLE patients, 170 adult-onset SLE (aSLE) patients and 200 healthy individuals were evaluated for C4A and C4B GCN by quantitative real-time PCR. RESULTS: JSLE patients had lower GCN for C4A (mean = 1...
May 2016: Rheumatology
https://www.readbyqxmd.com/read/26581448/successful-control-of-juvenile-dermatomyositis-associated-macrophage-activation-syndrome-and-interstitial-pneumonia-distinct-kinetics-of-interleukin-6-and-18-levels
#17
Hiroyuki Wakiguchi, Shunji Hasegawa, Reiji Hirano, Hidenobu Kaneyasu, Midori Wakabayashi-Takahara, Shouichi Ohga
BACKGROUND: Macrophage activation syndrome (MAS) is the secondary hemophagocytic lymphohistiocytosis associated with rheumatic diseases. Recently, the different cytokine profiles between systemic juvenile idiopathic arthritis (sJIA)-associated MAS (sJIA-MAS) and juvenile systemic lupus erythematosus (JSLE)-associated MAS (JSLE-MAS) were reported. However, there is little information about juvenile dermatomyositis (JDM)-associated MAS (JDM-MAS). CASE PRESENTATION: A 4-year-old girl with JDM was hospitalized because of fever, erythema, hepatosplenomegaly, cytopenia, liver dysfunction and coagulopathy...
November 18, 2015: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/26453665/increased-expression-of-low-density-granulocytes-in-juvenile-onset-systemic-lupus-erythematosus-patients-correlates-with-disease-activity
#18
A Midgley, M W Beresford
Neutrophils are implicated in a wide range of non-infectious inflammatory conditions. A subset of neutrophils in the peripheral circulation of systemic lupus erythematosus (SLE) patients has been described and termed low density granulocytes (LDGs). This study investigates the expression of LDG in juvenile-onset SLE (JSLE) patients compared to controls, and any correlations with disease activity.Neutrophils and LDGs were isolated from JSLE (n = 13) and paediatric non-inflammatory control patients (n = 12)...
April 2016: Lupus
https://www.readbyqxmd.com/read/26337941/anti-cd20-rituximab-therapy-in-refractory-pediatric-rheumatic-diseases
#19
Joel Reis, Francisca Aguiar, Iva Brito
OBJECTIVES: We aim to report the efficacy and safety of rituximab (RTX) in patients diagnosed with juvenile systemic lupus erythematosus (JSLE) or juvenile idiopathic arthritis (JIA) refractory to conventional treatment. METHODS: A retrospective review was made of all medical records of patients with JSLE or JIA treated with RTX between January 2009 and January 2015 in the Pediatric Rheumatology Unit of a central hospital. RESULTS: Five patients, 4 with JSLE and 1 with extended oligoarticular JIA, received 10 cycles of RTX (23 infusions)...
August 3, 2015: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/26335858/-juvenile-systemic-lupus-erythematosus-with-unusual-manifestation-of-lupus-associated-panniculitis
#20
H Hashemie, N Klossowski, P T Oommen, J Neubert, B Homey, N-P Hoff, J Reifenberger, S Meller
Juvenile systemic lupus erythematosus (JSLE) is a rare multisystem autoimmune disease with broad heterogeneity of clinical manifestations. Diagnosing JSLE is often very challenging. This life-threatening, unpredictable, and relapsing disease, which may affect various organ systems, requires interdisciplinary, lifelong care. Here, we report the case of a 13-year-old patient with JSLE suffering from recurrent arthralgia, lupus panniculitis, and rashes that were successfully treated with hydroxychloroquine and prednisolone...
October 2015: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
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