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1دورية أكاديمية
المؤلفون: Nguyen, Huong D, Jouen, Fabienne, Déchelotte, Benoit, Cordel, Nadège, Gitiaux, Cyril, Bodemer, Christine, Quartier, Pierre, Belot, Alexandre, O’Brien, Kathryn, Cancemi, Dario, Melki, Isabelle, Fabien, Nicole, Tansley, Sarah, Boyer, Olivier, Wedderburn, Lucy R, Bader-Meunier, Brigitte, Armon, Kate, Coke, Louise, Cook, Julie, Nichols, Amy, McCann, Liza, Roberts, Ian, Baildam, Eileen, Hanna, Louise, Lloyd, Olivia, Wadeson, Susan, Andrews, Michelle, Riley, Phil, McGovern, Ann, Cuthbert, Verna, Ryder, Clive, Scott, Janis, Thomas, Beverley, Southwood, Taunton, Al-Abadi, Eslam, Howman, Ruth, Wyatt, Sue, Jackson, Gillian, Wood, Mark, Amin, Tania, VanRooyen, Vanessa, Burton, Deborah, Turner, Louise, Rostron, Heather, Hanson, Sarah, Davidson, Joyce, Gardner-Medwin, Janet, Martin, Neil, Ferguson, Sue, Waxman, Liz
المساهمون: UK JDM Cohort, Biomarker study, Wellcome Trust, Action Medical Research, Myositis Support Group, Arthritis Research, Versus Arthritis, Henry Smith Charity, Great Ormond Street Children’s Charity, Tiny Hearts Society, Remission Charity, Myositis Association, Cure JM Foundation, Medical Research Council, National Institute for Health Research
المصدر: Rheumatology ; ISSN 1462-0324 1462-0332
مصطلحات موضوعية: Pharmacology (medical), Rheumatology
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2دورية أكاديمية
المؤلفون: Md Yusof, Md Yuzaiful, Smith, Eve M.D., Ainsworth, Sammy, Armon, Kate, Beresford, Michael W., Brown, Morgan, Cherry, Lindsey, Edwards, Christopher J., Flora, Kalveer, Gilman, Rebecca, Griffiths, Bridget, Gordon, Caroline, Howard, Paul, Isenberg, David, Jordan, Natasha, Kaul, Arvind, Lanyon, Peter, Laws, Philip M., Lightsone, Liz, Lythgoe, Hanna, Mallen, Christian D., Marks, Stephen D., Maxwell, Naomi, Moraitis, Elena, Nash, Clare, Pepper, Ruth J., Pilkington, Clarissa, Psarras, Antonios, Rostron, Heather, Skeates, Jade, Skeoch, Sarah, Tremarias, Dalila, Wincup, Chris, Zoma, Asad, Vital, Edward M.
الوصف: The objective of this guideline is to provide up-to-date, evidence-based recommendations for the management of SLE that builds upon the existing treatment guideline for adults living with SLE published in 2017. This will incorporate advances in the assessment, diagnosis, monitoring, non-pharmacological and pharmacological management of SLE. General approaches to management as well as organ-specific treatment, including lupus nephritis and cutaneous lupus, will be covered. This will be the first guideline in SLE using a whole life course approach from childhood through adolescence and adulthood. The guideline will be developed with people with SLE as an important target audience in addition to healthcare professionals. It will include guidance related to emerging approved therapies and account for National Institute for Health and Care Excellence Technology Appraisals, National Health Service England clinical commissioning policies and national guidance relevant to SLE. The guideline will be developed using the methods and rigorous processes outlined in ‘Creating Clinical Guidelines: Our Protocol’ by the British Society for Rheumatology.
وصف الملف: text
العلاقة: https://eprints.soton.ac.uk/485845/1/rkad093.pdfTest; Md Yusof, Md Yuzaiful, Smith, Eve M.D., Ainsworth, Sammy, Armon, Kate, Beresford, Michael W., Brown, Morgan, Cherry, Lindsey, Edwards, Christopher J., Flora, Kalveer, Gilman, Rebecca, Griffiths, Bridget, Gordon, Caroline, Howard, Paul, Isenberg, David, Jordan, Natasha, Kaul, Arvind, Lanyon, Peter, Laws, Philip M., Lightsone, Liz, Lythgoe, Hanna, Mallen, Christian D., Marks, Stephen D., Maxwell, Naomi, Moraitis, Elena, Nash, Clare, Pepper, Ruth J., Pilkington, Clarissa, Psarras, Antonios, Rostron, Heather, Skeates, Jade, Skeoch, Sarah, Tremarias, Dalila, Wincup, Chris, Zoma, Asad and Vital, Edward M. (2023) Management and treatment of children, young people and adults with systemic lupus erythematosus: British Society for Rheumatology guideline scope. Rheumatology Advances in Practice, 7 (3), [rkad093]. (doi:10.1093/rap/rkad093 ).
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3دورية أكاديمية
المؤلفون: Vetsiou, Evangelia, Armon, Kate, Gass, Julia
المصدر: British Journal of Dermatology ; volume 190, issue Supplement_1, page i9-i9 ; ISSN 0007-0963 1365-2133
مصطلحات موضوعية: Dermatology
الوصف: A 15-year-old female with skin of colour (SOC), was diagnosed with Juvenile dermatomyositis (JDM) following a presentation with muscle weakness, swallowing difficulties and rash. Investigations showed CK>1300, positive Mi-2 alpha, beta antibodies and bilateral myositis on MRI pelvis. Her skin features commenced 2 months prior to her rheumatological presentation. They included erythematous pruritic patches on the limbs, generalised hyperpigmentation of the face and trunk, hyperpigmentation of palmar creases and nail folds, flagellate hyperpigmentation of the hands & arms, and hypopigmented macules with follicular sparing across the face. She also presented skin thickening over the interphalangeal joints and nail fold capillary dilatation and infarctions. Skin biopsy showed interface dermatitis, in keeping with dermatomyositis. She was managed with weaning prednisolone, methotrexate, topical tacrolimus and sunscreen. JDM is a small-vessel autoimmune vasculopathy leading to muscle inflammation and skin changes. Unlike adult-onset disease, JDM is not associated with malignancy. The typically described violaceous skin features of JDM including photosensitive rash periorbitally, and over the knees and knuckles (Gottron’s sign) may be hyperpigmented rather than erythematous in skin of colour. Additional skin features described in SOC are generalised hyperpigmentation and pigmentation in skin creases. A flagellate dermatitis is uncommon, but recognised in association with Mi-2 antibodies in JDM. TIF1 antibodies have been reported in association with hypopigmented patches which may mimic vitiligo postulated possibly secondary to interface inflammation perhaps leading to dyspigmnentation. Notably our patient was negative for TIF1. This presentation highlights some of the less commonly described skin features of dyspigmentation in JDM in SOC.
الإتاحة: https://doi.org/10.1093/bjd/ljad483.024Test
https://academic.oup.com/bjd/article-pdf/190/Supplement_1/i9/54898971/ljad483.024.pdfTest -
4دورية أكاديمية
المؤلفون: Price-Kuehne, Fiona, Armon, Kate
المصدر: Current Pediatric Reviews ; volume 20, issue 4, page 405-411 ; ISSN 1573-3963
الوصف: Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disorder mostly affecting children and adolescents. Although it is considered a rare disease, CNO is likely to be the single most common autoinflammatory bone disease in childhood, underdiagnosed and underreported due to a lack of awareness of the condition in both medics and patients and the absence of validated diagnostic criteria. The exact underlying pathogenesis of CNO remains unknown, making targeted treatment difficult. This issue is exacerbated by the lack of any randomised control trials, meaning that treatment strategies are based solely on retrospective reviews and case series. : This review summarises the current concepts in pathophysiology, the clinical features that help differentiate important differential diagnoses, and an approach to investigating and managing children with CNO. Ultimately, the timely and thorough investigation of children and young people with CNO is vitally important to exclude important mimics and initiate appropriate management that can prevent the complications of persistent inflammatory bone disease.
الإتاحة: https://doi.org/10.2174/1573396319666221027123723Test
https://www.eurekaselect.com/210418/articleTest -
5دورية أكاديمية
المؤلفون: Smith, Eve M. D., Egbivwie, Naomi, Jorgensen, Andrea L., Ciurtin, Coziana, Al-Abadi, Eslam, Armon, Kate, Bailey, Kathryn, Brennan, Mary, Gardner-Medwin, Janet, Haslam, Kirsty, Hawley, Daniel P., Leahy, Alice, Leone, Valentina, Malik, Gulshan, McLaren, Zoe, Pilkington, Clarissa, Ramanan, Athimalaipet V., Rangaraj, Satyapal, Ratcliffe, Annie, Riley, Phil, Sen, Ethan, Sridhar, Arani, Wilkinson, Nick, Wood, Fiona, Beresford, Michael W., Hedrich, Christian M.
الوصف: Background: In the absence of clinical trials evidence, Juvenile-onset Systemic Lupus Erythematosus (JSLE) treatment plans vary. Aim: To explore ‘real world’ treatment utilising longitudinal UK JSLE Cohort Study data. Methods: Data collected between 07/2009–05/2020 was used to explore the choice/sequence of immunomodulating drugs from diagnosis. Multivariate logistic regression determined how organ-domain involvement (pBILAG-2004) impacted treatment choice. Result: 349 patients met inclusion criteria, median follow-up 4-years (IQR:2,6). Mycophenolate mofetil (MMF) was most commonly used for the majority of organ-domains, and significantly associated with renal involvement (OR:1.99, 95% CI:1.65–2.41, pc < 0.01). Analyses assessing the sequence of immunomodulators focused on 197/349 patients (meeting relevant inclusion/exclusion criteria). 10/197 (5%) solely recieved hydroxychloroquine/prednisolone, 62/197 (31%) received a single-immunomodulator, 69/197 (36%) received two, and 36/197 patients (28%) received ≥three immunomodulators. The most common first and second line immunomodulator was MMF. Rituximab was the most common third-line immunomodulator. Conclusions: Most UK JSLE patients required ≥two immunomodulators, with MMF used most commonly.
وصف الملف: text
العلاقة: https://eprints.gla.ac.uk/271283/1/271283.pdfTest; Smith, E. M. D. et al. (2022) Real world treatment of juvenile-onset systemic lupus erythematosus: Data from the UK JSLE cohort study. Clinical Immunology , 239, 109028. (doi:10.1016/j.clim.2022.109028 ) (PMID:35513304)
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6دورية أكاديمية
المؤلفون: Russell, Mark D, Dey, Mrinalini, Flint, Julia, Davie, Philippa, Allen, Alexander, Crossley, Amy, Frishman, Margreta, Gayed, Mary, Hodson, Kenneth, Khamashta, Munther, Moore, Louise, Panchal, Sonia, Piper, Madeleine, Reid, Clare, Saxby, Katherine, Schreiber, Karen, Senvar, Naz, Tosounidou, Sofia, van de Venne, Maud, Warburton, Louise, Williams, David, Yee, Chee-Seng, Gordon, Caroline, Giles, Ian, Roddy, Ed, Armon, Kate, Astell, Lauren, Cotton, Caroline, Davidson, Alan, Fordham, Sarah, Jones, Claire, Joyce, Christopher, Kuttikat, Anoop, McLaren, Zoe, Merrison, Karen, Mewar, Devesh, Mootoo, Amanda, Williams, Emma
المصدر: Rheumatology ; volume 62, issue 4, page e48-e88 ; ISSN 1462-0324 1462-0332
الإتاحة: https://doi.org/10.1093/rheumatology/keac551Test
https://academic.oup.com/rheumatology/article-pdf/62/4/e48/49750710/keac551.pdfTest -
7دورية أكاديمية
المؤلفون: Schreiber, Karen, Frishman, Margreta, Russell, Mark D, Dey, Mrinalini, Flint, Julia, Allen, Alexander, Crossley, Amy, Gayed, Mary, Hodson, Kenneth, Khamashta, Munther, Moore, Louise, Panchal, Sonia, Piper, Madeleine, Reid, Clare, Saxby, Katherine, Senvar, Naz, Tosounidou, Sofia, van de Venne, Maud, Warburton, Louise, Williams, David, Yee, Chee-Seng, Gordon, Caroline, Giles, Ian, Roddy, Ed, Armon, Kate, Astell, Lauren, Cotton, Caroline, Davidson, Alan, Fordham, Sarah, Jones, Claire, Joyce, Christopher, Kuttikat, Anoop, McLaren, Zoe, Merrison, Karen, Mewar, Devesh, Mootoo, Amanda, Williams, Emma
المصدر: Rheumatology ; volume 62, issue 4, page 1388-1397 ; ISSN 1462-0324 1462-0332
الإتاحة: https://doi.org/10.1093/rheumatology/keac559Test
https://academic.oup.com/rheumatology/article-pdf/62/4/1388/49750835/keac559.pdfTest -
8دورية أكاديمية
المؤلفون: Schreiber, Karen, Frishman, Margreta, Russell, Mark D, Dey, Mrinalini, Flint, Julia, Allen, Alexander, Crossley, Amy, Gayed, Mary, Hodson, Kenneth, Khamashta, Munther, Moore, Louise, Panchal, Sonia, Piper, Madeleine, Reid, Clare, Saxby, Katherine, Senvar, Naz, Tosounidou, Sofia, van de Venne, Maud, Warburton, Louise, Williams, David, Yee, Chee-Seng, Gordon, Caroline, Giles, Ian, Roddy, Ed, Armon, Kate, Astell, Lauren, Cotton, Caroline, Davidson, Alan, Fordham, Sarah, Jones, Claire, Joyce, Christopher, Kuttikat, Anoop, McLaren, Zoe, Merrison, Karen, Mewar, Devesh, Mootoo, Amanda, Williams, Emma
المصدر: Rheumatology ; volume 62, issue 4, page e89-e104 ; ISSN 1462-0324 1462-0332
الإتاحة: https://doi.org/10.1093/rheumatology/keac552Test
https://academic.oup.com/rheumatology/article-pdf/62/4/e89/49750475/keac552.pdfTest -
9دورية أكاديمية
المؤلفون: Russell, Mark D, Dey, Mrinalini, Flint, Julia, Davie, Philippa, Allen, Alexander, Crossley, Amy, Frishman, Margreta, Gayed, Mary, Hodson, Kenneth, Khamashta, Munther, Moore, Louise, Panchal, Sonia, Piper, Madeleine, Reid, Clare, Saxby, Katherine, Schreiber, Karen, Senvar, Naz, Tosounidou, Sofia, van de Venne, Maud, Warburton, Louise, Williams, David, Yee, Chee-Seng, Gordon, Caroline, Giles, Ian, Roddy, Ed, Armon, Kate, Astell, Lauren, Cotton, Caroline, Davidson, Alan, Fordham, Sarah, Jones, Claire, Joyce, Christopher, Kuttikat, Anoop, McLaren, Zoe, Merrison, Karen, Mewar, Devesh, Mootoo, Amanda, Williams, Emma
المصدر: Rheumatology ; volume 62, issue 4, page 1370-1387 ; ISSN 1462-0324 1462-0332
الإتاحة: https://doi.org/10.1093/rheumatology/keac558Test
https://academic.oup.com/rheumatology/article-pdf/62/4/1370/49750175/keac558.pdfTest -
10دورية أكاديمية
المؤلفون: Charras, Amandine, Haldenby, Sam, Smith, Eve M D, Egbivwie, Naomi, Olohan, Lisa, Kenny, John G, Schwarz, Klaus, Roberts, Carla, Al-Abadi, Eslam, Armon, Kate, Bailey, Kathryn, Ciurtin, Coziana, Gardner-Medwin, Janet, Haslam, Kirsty, Hawley, Daniel P, Leahy, Alice, Leone, Valentina, McErlane, Flora, Modgil, Gita, Pilkington, Clarissa, Ramanan, Athimalaipet V, Rangaraj, Satyapal, Riley, Phil, Sridhar, Arani, Beresford, Michael W, Hedrich, Christian M
المساهمون: LUPUS, UK’s Experimental Arthritis Treatment Centre for Children, Alder Hey Children’s NHS Foundation Trust, University of Liverpool, NIHR
المصدر: Rheumatology ; volume 62, issue SI2, page SI210-SI225 ; ISSN 1462-0324 1462-0332
مصطلحات موضوعية: Pharmacology (medical), Rheumatology
الوصف: Objectives Juvenile-onset systemic lupus erythematosus (jSLE) affects 15–20% of lupus patients. Clinical heterogeneity between racial groups, age groups and individual patients suggests variable pathophysiology. This study aimed to identify highly penetrant damaging mutations in genes associated with SLE/SLE-like disease in a large national cohort (UK JSLE Cohort Study) and compare demographic, clinical and laboratory features in patient sub-cohorts with ‘genetic’ SLE vs remaining SLE patients. Methods Based on a sequencing panel designed in 2018, target enrichment and next-generation sequencing were performed in 348 patients to identify damaging gene variants. Findings were integrated with demographic, clinical and treatment related datasets. Results Damaging gene variants were identified in ∼3.5% of jSLE patients. When compared with the remaining cohort, ‘genetic’ SLE affected younger children and more Black African/Caribbean patients. ‘Genetic’ SLE patients exhibited less organ involvement and damage, and neuropsychiatric involvement developed over time. Less aggressive first line treatment was chosen in ‘genetic’ SLE patients, but more second and third line agents were used. ‘Genetic’ SLE associated with anti-dsDNA antibody positivity at diagnosis and reduced ANA, anti-LA and anti-Sm antibody positivity at last visit. Conclusion Approximately 3.5% of jSLE patients present damaging gene variants associated with younger age at onset, and distinct clinical features. As less commonly observed after treatment induction, in ‘genetic’ SLE, autoantibody positivity may be the result of tissue damage and explain reduced immune complex-mediated renal and haematological involvement. Routine sequencing could allow for patient stratification, risk assessment and target-directed treatment, thereby increasing efficacy and reducing toxicity.
الإتاحة: https://doi.org/10.1093/rheumatology/keac275Test
https://academic.oup.com/rheumatology/article-pdf/62/SI2/SI210/49318842/keac275.pdfTest
