journal
MENU ▼
Read by QxMD icon Read
search

Best Practice & Research. Clinical Rheumatology

journal
https://www.readbyqxmd.com/read/29773275/periodic-fever-syndromes
#1
REVIEW
Helen J Lachmann
Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the absence of autoantibody production or identifiable infection. The best recognised disorders include CAPS, FMF, TRAPS and MKD. Understanding the molecular pathogenesis of these disorders provides unique insights into the regulation of innate immunity. Diagnosis relies on clinical acumen and is supported by genetic testing...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773274/scleroderma-in-children
#2
REVIEW
Francesco Zulian
Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773273/childhood-systemic-vasculitis
#3
REVIEW
Erdal Sag, Ezgi Deniz Batu, Seza Ozen
Vasculitides are characterized by inflammation of the vessel wall. Most of the vasculitides tend to occur in vessels of a specific size and certain target organs. In this review, we discuss each specific childhood vasculitis according to the latest Chapel Hill Consensus Conference 2012 nomenclature system and the Ankara 2008 classification criteria. We have also reviewed the clinical and laboratory characteristics and the recent treatment recommendations for the vasculitides we encounter in children.
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773272/juvenile-dermatomyositis-latest-advances
#4
REVIEW
Qiong Wu, Lucy R Wedderburn, Liza J McCann
Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773271/juvenile-idiopathic-arthritis-associated-uveitis
#5
REVIEW
Ethan S Sen, A V Ramanan
Juvenile idiopathic arthritis (JIA) is the commonest rheumatic disease in children and JIA-associated uveitis its most frequent extra-articular manifestation. The uveitis is potentially sight-threatening and thus carries a considerable risk of morbidity with associated reduction in quality of life. The commonest form of uveitis seen in association with JIA is chronic anterior uveitis, which is almost always asymptomatic in the initial stages. Therefore, screening for JIA-associated uveitis in at-risk patients is essential...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773270/systemic-juvenile-idiopathic-arthritis-new-insights-into-pathogenesis-and-cytokine-directed-therapies
#6
REVIEW
Manuela Pardeo, Claudia Bracaglia, Fabrizio De Benedetti
Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773269/juvenile-onset-systemic-lupus-erythematosus-jsle-pathophysiological-concepts-and-treatment-options
#7
REVIEW
Christian M Hedrich, Eve M D Smith, Michael W Beresford
The systemic autoimmune/inflammatory condition systemic lupus erythematosus (SLE) manifests before the age of 16 years in 10-20% of all cases. Clinical courses are more severe, and organ complications are more common in patients with juvenile SLE. Varying gender distribution in different age groups and increasing severity with younger age and the presence of monogenic disease in early childhood indicate distinct differences in the pathophysiology of juvenile versus adult-onset SLE. Regardless of these differences, classification criteria and treatment options are identical...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773268/recent-therapeutic-advances-in-juvenile-idiopathic-arthritis
#8
REVIEW
Gabriella Giancane, Alessandra Alongi, Silvia Rosina, Jessica Tibaldi, Alessandro Consolaro, Angelo Ravelli
Over the past two decades, the management of juvenile idiopathic arthritis (JIA) has been revolutionized by the increased tendency toward early aggressive interventions and the availability of the novel biologic medications. In 2017, three novel randomized controlled trials have evaluated the effectiveness and tolerability of golimumab and tocilizumab in polyarticular JIA, and shown that methotrexate may increase and prolong the effect of intra-articular corticosteroid injection in children with oligoarthritis...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773267/update-on-research-and-clinical-translation-on-specific-clinical-areas-from-biology-to-bedside-unpacking-the-mysteries-of-juvenile-idiopathic-arthritis-pathogenesis
#9
REVIEW
Jorg van Loosdregt, Femke van Wijk, Berent Prakken, Bas Vastert
In the past decades, we have gained important insights into the mechanisms of disease and therapy underlying chronic inflammation in juvenile idiopathic arthritis (JIA). These insights have resulted in several game-changing therapeutic modalities for many patients. However, additional progress still has to be made with regard to efficacy, cost reduction, minimization of side effects, and dose-tapering and stop strategies of maintenance drugs. Moreover, to really transform the current therapeutic strategies into personalized medicine, we need validated biomarkers to translate increased insights into clinical practice...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773266/genetic-interferonopathies-an-overview
#10
REVIEW
Despina Eleftheriou, Paul A Brogan
Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses. Although differing in the degree of phenotypic expression and severity, the clinical presentation of these diseases shows a considerable degree of overlap, reflecting their common pathogenetic mechanisms. Increased understanding of the molecular basis of these Mendelian disorders has led to the identification of targeted therapies for these diseases, which could also be of potential relevance for non-genetic IFN-mediated diseases such as systemic lupus erythematosus and juvenile dermatomyositis...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773265/preface-recent-advances-in-autoimmune-and-auto-inflammatory-diseases-in-childhood
#11
EDITORIAL
A V Ramanan, M W Beresford
No abstract text is available yet for this article.
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224682/calcineurin-inhibitors-in-systemic-lupus-erythematosus
#12
REVIEW
Chi Chiu Mok
The calcineurin inhibitors (CNIs) belong to a group of immunosuppressive agents that block T-cell activation through the suppression of the calcium/calcimodulin-dependent phosphatase calcineurin. Agents such as cyclosporine A (CSA) and tacrolimus (TAC) have long been used in patients with systemic lupus erythematosus (SLE). TAC is preferred to CSA in SLE because of the lower frequency of cosmetic, hypertensive and dyslipidemic adverse effects. Recent randomised controlled trials have demonstrated noninferiority of TAC to mycophenolate mofetil (MMF) or cyclophosphamide (CYC) for induction therapy of lupus nephritis...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224681/role-of-interferons-in-sle
#13
REVIEW
Anders A Bengtsson, Lars Rönnblom
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that affects many different organ systems, with excessive production of type I interferons (IFNs) and autoantibodies against nucleic acids as hallmarks. Activation of the type I IFN system in SLE is due to continuous stimulation of plasmacytoid dendritic cells by endogenous nucleic acids, leading to sustained type I IFN production. This is reflected by an overexpression of type I IFN-regulated genes or an IFN signature. Type I IFNs have effects on both the innate and adaptive immune systems, which contribute to both loss of tolerance and the autoimmune disease process...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224680/pregnancy-in-systemic-lupus-erythematosus-and-antiphospholipid-syndrome
#14
REVIEW
Rebecca Fischer-Betz, Christof Specker
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with a high prevalence in females of childbearing age. Pregnancy in SLE nowadays has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, and preterm birth are well-known risks in such pregnancies. Anti-SS-A(Ro)/SS-B(La) antibodies put fetuses at risk for congenital heart block and neonatal lupus. Several risk factors for adverse pregnancy outcomes have been identified. Women with antiphospholipid antibodies or antiphospholipid syndrome and lupus nephritis represent a group with high risk for obstetric complications...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224679/a-review-on-sle-and-malignancy
#15
REVIEW
May Y Choi, Kelsey Flood, Sasha Bernatsky, Rosalind Ramsey-Goldman, Ann E Clarke
Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease characterized by autoantibody production, complement activation, and immune complex deposition. It predominantly affects young and middle-aged women. While improvements in the diagnosis and treatment of SLE have altered prognosis, morbidity and mortality rates remain higher than the general population. In addition to renal injury, cardiovascular disease, and infection, malignancy is known to be a significant cause of death in this population...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224678/atherosclerosis-in-systemic-lupus-erythematosus
#16
REVIEW
Sara Croca, Anisur Rahman
Cardiovascular disease (CVD), comprising coronary heart disease and stroke, is one of the most important causes of death in patients with systemic lupus erythematosus (SLE). The risks of developing both clinical CVD and sub-clinical atherosclerosis are increased in patients with SLE. This increase is not fully explained by traditional cardiovascular risk factors such as smoking, hypertension and elevated cholesterol, and it is believed that immune dysfunction also contributes to CVD risk in SLE. In particular, recent studies have shown that abnormalities in both serum lipid profile and the autoantibody and T lymphocyte response to lipids may play a role in development of atherosclerosis...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224677/diagnostic-and-prognostic-tests-in-systemic-lupus-erythematosus
#17
REVIEW
Natalia Vasquez-Canizares, Dawn Wahezi, Chaim Putterman
Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease characterized by autoantibodies directed against numerous self-nuclear antigens. Because of the heterogeneous nature of lupus, it has been challenging to identify markers that are sensitive and specific enough for its diagnosis and monitoring. However, with the sequencing of the human genome, rapid development of high-throughput approaches has allowed for a better understanding of the etiopathogenesis of complex diseases, including SLE...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224676/treat-to-target-remission-and-low-disease-activity-in-sle
#18
REVIEW
Eric F Morand, Marta Mosca
Despite improvements in survival, outcomes of contemporary treatment of systemic lupus erythematosus (SLE) are unacceptable. Unlike in many diseases, treat-to-target (T2T) approaches have not been adopted in SLE, owing to a lack of validated targets to treat towards. Therefore, it is a key goal to validate target state definitions such as low disease activity and remission, and test their implementation in clinical practice and clinical trials. In this article, we review recent advances in T2T approaches in SLE, and emerging evidence-based consensus on definitions of remission and low disease activity that are needed to underpin such approaches...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224675/mechanisms-of-thrombosis-in-systemic-lupus-erythematosus-and-antiphospholipid-syndrome
#19
REVIEW
Philip G de Groot, Bas de Laat
The presence of antiphospholipid antibodies is one of the most common acquired risk factors for thrombosis. Antiphospholipid antibodies is a collective term for a set of autoantibodies with closely related but different specificity. Experiments in which isolated patient antibodies were injected into mice have shown that a specific subset of autoantibodies, those directed against the first domain of plasma protein β2 -glycoprotein I, can explain the increased risk of thrombosis. Experiments performed with these mice have shown that autoantibodies against β2 -glycoprotein I bind to and activate cells such as endothelial cells, monocytes, and platelets...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29224674/drivers-of-the-immunopathogenesis-in-systemic-lupus-erythematosus
#20
REVIEW
Thomas Rose, Thomas Dörner
This review summarises a number of current insights into the pathogenesis of SLE. On the basis of the interaction of environmental factors within a predisposed host, a chronic autoimmune process gains function with a positive feed-forward loop between innate and adaptive immunity. A current focus of SLE pathogenesis is on the enhanced production of certain cytokines, such as type I interferons and BLyS/BAFF, suggesting continuous plasmacytoid dendritic and myeloid cell activity together with disturbances of B lineage cells (increased autoantibodies, including anti-dsDNA and plasmablasts, which correlate with SLE activity and memory B-cell abnormalities)...
June 2017: Best Practice & Research. Clinical Rheumatology
journal
journal
39746
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"