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juvenile systemic lupus

Eve M D Smith, Peng Yin, Andrea L Jorgensen, Michael W Beresford
BACKGROUND: Proteinuria is a well-known risk factor for progression of renal dysfunction in a variety of chronic kidney diseases. In adult-onset Systemic Lupus Erytematosus (SLE) patients with lupus nephritis (LN), proteinuria takes a significant period of time to normalise, with proteinuric remission being associated with improved renal survival and reductions in mortality. The length of time required to attain proteinuric remission has not previously been investigated in Juvenile-onset SLE (JSLE)...
February 21, 2018: Pediatric Rheumatology Online Journal
H H Abdel-Nabi, R A Abdel-Noor
Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease that occurs worldwide in both children and adults, with different disease manifestations, activity and severity between them. Objectives To analyse the difference in disease onset patterns and activity in Egyptian children and adults with SLE. Methods A retrospective cohort study conducted on 298 Egyptian SLE patients, 215 adults (a-SLE) (>18 years) and 83 children (j-SLE) (≤18 years). Disease onset, clinical manifestations and laboratory investigations were recorded...
January 1, 2018: Lupus
Catherine G Anelli, Claudio A Len, Maria T Terreri, Gleice C S Russo, Andreas O Reiff
OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool...
February 16, 2018: Jornal de Pediatria
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
K T El Hadidi, B M Medhat, N M Abdel Baki, H Abdel Kafy, W Abdelrahaman, A Y Yousri, D H Attia, M Eissa, D El Dessouki, I Elgazzar, F T Elgengehy, N El Ghobashy, H El Hadary, G El Mardenly, H El Naggar, A M El Nahas, R M El Refai, H Allah El Rwiny, R M Elsman, M Galal, S Ghoniem, L A Maged, S M Sally, H Naji, S Saad, M Shaaban, M Sharaf, N Sobhy, R M Soliman, T S El Hadidi
Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can vary among different ethnic and racial groups. Objective The objective of this paper is to study the prevalence of various manifestations of SLE in a sample of the Egyptian population. Patients and methods Information in this study was derived from the medical records of SLE patients who sought medical advice at a private clinic in Cairo from January 1980 to June 2016. Results This study included 1109 SLE patients, of whom 114 (10...
January 1, 2018: Lupus
Yusuke Takeuchi, Tomoko Seki, Norimoto Kobayashi, Kenji Sano, Tomonari Shigemura, Hisashi Shimojo, Kazuhiko Matsumoto, Kazunaga Agematsu
No abstract text is available yet for this article.
February 1, 2018: Modern Rheumatology
Surupa Basu, Biplab Maji, Santanu Barman, Apurba Ghosh
Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included...
January 2018: Indian Journal of Clinical Biochemistry: IJCB
Esther A R Hartman, Annet van Royen-Kerkhof, Johannes W G Jacobs, Paco M J Welsing, Ruth D E Fritsch-Stork
OBJECTIVE: To evaluate the performance in classifying systemic lupus erythematosus by the 2012 Systemic Lupus International Collaborating Clinics criteria (SLICC'12), versus the revised American College of Rheumatology criteria from 1997 (ACR'97) in adult and juvenile SLE patients. METHODS: A systematic literature search was conducted in PubMed and Embase for studies comparing SLICC'12 and ACR'97 with clinical diagnosis. A meta-analysis was performed to estimate the sensitivity and specificity of SLICC'12 and ACR'97...
January 21, 2018: Autoimmunity Reviews
Stefano Stagi, Donato Rigante
Systemic lupus erythematosus (SLE) and juvenile SLE (jSLE) are autoimmune disorders naturally associated with several genetic, environmental, hormonal, and immunological contributing factors. It has been assumed that vitamin D deficiency may have a role in the immune activation of patients with SLE and play an active part in many comorbidities and even complications. A host of clinical studies suggested that vitamin D exerts inhibitory effects on many immunological abnormalities associated with SLE, also in children and adolescents, while different reports have hypothesized that vitamin D may be associated with accelerated cardiovascular disease in SLE...
January 15, 2018: Autoimmunity Reviews
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
S G L da Silva, M T Terreri, T T O Abad, D Machado, F L A Fonseca, S Hix, F I Suano-Souza, R O S Sarni, C A Len
Objective This study sought to evaluate the effects of a nutritional intervention on the lipid metabolism biomarkers associated with cardiovascular risk, and their variation over time, in juvenile systemic lupus erythematosus (JSLE) patients. This study also investigated the relationships between these biomarkers and dietary intake, nutritional status, disease variables, and medication used. Methods A total of 31 10- to 19-year-old female adolescents with JSLE for at least six months were analyzed. The participants were randomly allocated to two groups: nutritional intervention or control...
January 1, 2018: Lupus
Arash Salmaninejad, Mahdi Mahmoudi, Saeed Aslani, Shiva Poursani, Vahid Ziaee, Nima Rezaei
Salmaninejad A, Mahmoudi M, Aslani S, Poursani S, Ziaee V, Rezaei N. Association of STAT4 gene single nucleotide polymorphisms with Iranian juvenile-onset systemic lupus erythematosus patients. Turk J Pediatr 2017; 59: 144-149. Juvenile-onset systemic lupus erythematosus (JSLE) is a complex autoimmune disease, characterized by multi-organ involvement. Single nucleotide polymorphisms (SNPs) of signal transducer and activator of transcription 4 (STAT4) gene have been reported to have relationship with the risk of several autoimmune diseases...
2017: Turkish Journal of Pediatrics
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
S Sahin, A Adrovic, K Barut, N Canpolat, Y Ozluk, I Kilicaslan, S Caliskan, L Sever, O Kasapcopur
Objectives This paper aims to assess in a retrospective fashion the clinical and laboratory features, severity and outcome of juvenile systemic lupus erythematosus (jSLE) from a referral center in Turkey. Methods We have included all jSLE patients ( n = 92) diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2004 and January 2017. Results The most prevalent clinical feature in our cohort was mucocutaneous manifestations (97.8%), followed by constitutional (81...
January 1, 2017: Lupus
Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge...
2017: Case Reports in Rheumatology
Mahdi Mahmoudi, Saeed Aslani, Elham Hamzeh, Vahid Ziaee, Shiva Poursani, Mohammad Hossein Nicknam, Nima Rezaei
INTRODUCTION: Juvenile-onset systemic lupus erythematosus is a multigenic autoimmune disorder. Polymorphisms of MECP2 gene have been reported to increase the risk of adult-onset SLE. In this study, we aimed to analyze if MECP2 gene polymorphisms could impress the proneness to JSLE in Iranian population. MATERIAL AND METHODS: Polymorphisms of MECP2 gene were genotyped in 50 Iranian JSLE patients and 426 matched healthy controls employing the real-time PCR allelic discrimination technique...
December 2017: Fetal and Pediatric Pathology
Deepa Hammaker, Gary S Firestein
PURPOSE OF REVIEW: Aberrant epigenetic changes in DNA methylation, histone marks, and noncoding RNA expression regulate the pathogenesis of many rheumatic diseases. The present article will review the recent advances in the epigenetic profile of inflammatory arthritis and discuss diagnostic biomarkers and potential therapeutic targets. RECENT FINDINGS: Methylation signatures of fibroblast-like synoviocytes not only distinguish rheumatoid arthritis (RA) and osteoarthritis (OA), but also early RA from late RA or juvenile idiopathic arthritis...
March 2018: Current Opinion in Rheumatology
G L Lima, J A Paupitz, N E Aikawa, J C Alvarenga, R M Rodrigues Pereira
In this randomized double-blind placebo-controlled 24-week trial, cholecalciferol supplementation at 50,000 IU/week effectively improved bone microarchitecture parameters in juvenile-onset systemic lupus erythematosus (JoSLE) patients, as assessed by high-resolution peripheral quantitative computed tomography (HR-pQCT) at tibia site. An increase in the trabecular number and a decrease in the trabecular separation were observed, suggesting that vitamin D supplementation may be recommended for JoSLE patients with its deficiency...
November 19, 2017: Osteoporosis International
Vânia Vieira Borba, Kassem Sharif, Yehuda Shoenfeld
Breast milk is not only a completely adapted nutrition source for the newborn but also an impressive array of immune-active molecules that afford protection against infections and shape mucosal immune responses. Decisive imprinting events might be modulated during the first months of life with potential health long-term effects, enhancing the importance of breastfeeding as a major influence on the immune system correct development and modifying disease susceptibility. The aim of this review was to clarify the link between breastfeeding and autoimmune diseases, inquiring the related mechanisms, based on data available in the literature...
January 2018: American Journal of Reproductive Immunology: AJRI
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