Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Rebecca Fischer-Betz, Christof SpeckerAbstractSystemic 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Index
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Calcineurin inhibitors in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Chi Chiu MokAbstractThe 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 demon...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Role of interferons in SLE
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Anders A. Bengtsson, Lars RönnblomAbstractSystemic 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-...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

A review on SLE and malignancy
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): May Y. Choi, Kelsey Flood, Sasha Bernatsky, Rosalind Ramsey-Goldman, Ann E. ClarkeAbstractSystemic 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...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Editorial Board/Aims and Scope
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Diagnostic and prognostic tests in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Natalia Vasquez-Canizares, Dawn Wahezi, Chaim PuttermanAbstractSystemic 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 unde...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

SLE redefined on the basis of molecular pathways
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Guillermo Barturen, Marta E. Alarcón-RiquelmeAbstractThe implementation of precision medicine requires the recruiting of patients in statistically enough numbers, the possibility of obtaining enough materials, and the integration of data from various platforms, which are all real limitations. These types of studies have been performed extensively in cancer but barely on systemic lupus erythematosus (SLE) or other rheumatic diseases.To consider the practical use of the information obtained from such stu...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Understanding the role of environmental factors in the development of systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Christine G. Parks, Aline de Souza Espindola Santos, Medha Barbhaiya, Karen H. CostenbaderAbstractSystemic lupus erythematosus (SLE) is a multisystem disease with a complex etiology. Its risk is higher among women, racial and ethnic minorities, and individuals with a family history of SLE or related autoimmune diseases. It is believed that genetic factors interact with environmental exposures throughout the lifespan to influence susceptibility to developing SLE. The strongest epidemiologic evidence exists for...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Drivers of the immunopathogenesis in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Thomas Rose, Thomas DörnerAbstractThis 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 a...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Mechanisms of thrombosis in systemic lupus erythematosus and antiphospholipid syndrome
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Philip G. de Groot, Bas de LaatAbstractThe 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Treat to target, remission and low disease activity in SLE
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Eric F. Morand, Marta MoscaAbstractDespite 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 rec...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Atherosclerosis in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Sara Croca, Anisur RahmanAbstractCardiovascular 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Richard Furie, Ricard Cervera (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Juvenile dermatomyositis: Latest advances
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Qiong Wu, Lucy R. Wedderburn, Liza J. McCannAbstractRegistries 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 re...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Periodic fever syndromes
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Helen J. LachmannAbstractPeriodic 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Childhood systemic vasculitis
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Erdal Sag, Ezgi Deniz Batu, Seza OzenAbstractVasculitides 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Scleroderma in children
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Francesco ZulianAbstractJuvenile 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 mul...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Index
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Editorial Board
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Preface: Recent advances in autoimmune and auto-inflammatory diseases in childhood
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): A.V. Ramanan, M.W. Beresford (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Genetic interferonopathies: An overview
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Despina Eleftheriou, Paul A. BroganAbstractInterferonopathies 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 i...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Update on research and clinical translation on specific clinical areas from biology to bedside: Unpacking the mysteries of juvenile idiopathic arthritis pathogenesis
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Jorg van Loosdregt, Femke van Wijk, Berent Prakken, Bas VastertAbstractIn 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 d...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Recent therapeutic advances in juvenile idiopathic arthritis
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Gabriella Giancane, Alessandra Alongi, Silvia Rosina, Jessica Tibaldi, Alessandro Consolaro, Angelo RavelliAbstractOver 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 methot...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Juvenile-onset systemic lupus erythematosus (jSLE) – Pathophysiological concepts and treatment options
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Christian M. Hedrich, Eve M.D. Smith, Michael W. BeresfordAbstractThe 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 patho...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Systemic juvenile idiopathic arthritis: New insights into pathogenesis and cytokine directed therapies
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Manuela Pardeo, Claudia Bracaglia, Fabrizio De BenedettiAbstractSystemic 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 f...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Juvenile idiopathic arthritis-associated uveitis
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Ethan S. Sen, A.V. RamananAbstractJuvenile 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-as...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Wnt signaling as target for the treatment of osteoarthritis
Publication date: Available online 14 April 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Alessandra Stampella, Silvia Monteagudo, Rik LoriesAbstractOsteoarthritis is a severe and common rheumatic and skeletal disease for which currently no specific drugs are available. The Wnt signaling pathway modulates key biological processes in development, growth, homeostasis, and disease, particularly in the joints and bone. Excessive activation of the Wnt signaling pathway in the articular cartilage has been clearly associated with the onset and severity of osteoarthritis. Hence, targeting Wnt signaling m...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Osteoarthritis pain: What are we learning from animal models?
Publication date: Available online 26 April 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Rachel E. Miller, Anne-Marie MalfaitAbstractAll experimental models of osteoarthritis (OA)-like joint damage are accompanied by behaviors indicative of pain. In experimental knee OA, evoked pain responses to exogenously applied stimuli suggest that animals become sensitized to mechanical stimuli. Neurobiological techniques including electrophysiology and in vivo calcium imaging confirm that joint damage is associated with mechanical stimuli through peripheral sensitization. Several mediators present in ...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

New imaging modalities to predict and evaluate osteoarthritis progression
Publication date: Available online 22 May 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Nima Hafezi-Nejad, Ali Guermazi, Shadpour Demehri, Frank W. RoemerAbstractIn this narrative review, we discuss the role of different imaging methods for the evaluation of progression of structural osteoarthritis. We will focus on the role of less commonly applied imaging modalities and imaging biomarkers that were introduced in recent years or on established methods that have evolved into more prominent positions in recent years. We will highlight findings from longitudinal studies that focused on structural o...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Biomaterial-assisted cell therapy in osteoarthritis: From mesenchymal stem cells to cell encapsulation
Publication date: Available online 2 June 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Fahd Hached, Claire Vinatier, Catherine Le Visage, Henri Gondé, Jérôme Guicheux, Gaël Grimandi, Aurélie Billon-ChabaudAbstractOsteoarthritis (OA) is a degenerative and inflammatory joint disease that affects the cartilage, subchondral bone, and joint tissues. Although current drug therapies can provide a degree of symptomatic relief from pain, they fail to prevent joint damage. Mesenchymal stem/stromal cells (MSCs) have generated significant interest in terms of medical applicat...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Gaps in appropriate use of treatment strategies in osteoarthritis
Publication date: Available online 19 June 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Kelli D. Allen, Yvonne M. Golightly, Daniel K. WhiteAbstractOptimal management of osteoarthritis (OA) requires a combination of therapies, with behavioral (e.g., exercise and weight management) and rehabilitative components at the core, accompanied by pharmacological treatments and, in later stages, consideration of joint replacement surgery. Although multiple sets of OA treatment guidelines have been developed, there are gaps in the implementation of these recommendations. Key areas of concern include the un...
Source: Best Practice and Research Clinical Rheumatology - July 10, 2018 Category: Rheumatology Source Type: research

Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Rebecca Fischer-Betz, Christof SpeckerAbstractSystemic 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Index
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Calcineurin inhibitors in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Chi Chiu MokAbstractThe 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 demon...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Role of interferons in SLE
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Anders A. Bengtsson, Lars RönnblomAbstractSystemic 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-...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

A review on SLE and malignancy
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): May Y. Choi, Kelsey Flood, Sasha Bernatsky, Rosalind Ramsey-Goldman, Ann E. ClarkeAbstractSystemic 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...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Editorial Board/Aims and Scope
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Diagnostic and prognostic tests in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Natalia Vasquez-Canizares, Dawn Wahezi, Chaim PuttermanAbstractSystemic 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 unde...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

SLE redefined on the basis of molecular pathways
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Guillermo Barturen, Marta E. Alarcón-RiquelmeAbstractThe implementation of precision medicine requires the recruiting of patients in statistically enough numbers, the possibility of obtaining enough materials, and the integration of data from various platforms, which are all real limitations. These types of studies have been performed extensively in cancer but barely on systemic lupus erythematosus (SLE) or other rheumatic diseases.To consider the practical use of the information obtained from such stu...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Understanding the role of environmental factors in the development of systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Christine G. Parks, Aline de Souza Espindola Santos, Medha Barbhaiya, Karen H. CostenbaderAbstractSystemic lupus erythematosus (SLE) is a multisystem disease with a complex etiology. Its risk is higher among women, racial and ethnic minorities, and individuals with a family history of SLE or related autoimmune diseases. It is believed that genetic factors interact with environmental exposures throughout the lifespan to influence susceptibility to developing SLE. The strongest epidemiologic evidence exists for...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Drivers of the immunopathogenesis in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Thomas Rose, Thomas DörnerAbstractThis 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 a...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Mechanisms of thrombosis in systemic lupus erythematosus and antiphospholipid syndrome
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Philip G. de Groot, Bas de LaatAbstractThe 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Treat to target, remission and low disease activity in SLE
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Eric F. Morand, Marta MoscaAbstractDespite 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 rec...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Atherosclerosis in systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Sara Croca, Anisur RahmanAbstractCardiovascular 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Systemic lupus erythematosus
Publication date: June 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 3Author(s): Richard Furie, Ricard Cervera (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Juvenile dermatomyositis: Latest advances
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Qiong Wu, Lucy R. Wedderburn, Liza J. McCannAbstractRegistries 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 re...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Periodic fever syndromes
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Helen J. LachmannAbstractPeriodic 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Childhood systemic vasculitis
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Erdal Sag, Ezgi Deniz Batu, Seza OzenAbstractVasculitides 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 ...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research

Scleroderma in children
Publication date: August 2017Source: Best Practice & Research Clinical Rheumatology, Volume 31, Issue 4Author(s): Francesco ZulianAbstractJuvenile 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 mul...
Source: Best Practice and Research Clinical Rheumatology - July 5, 2018 Category: Rheumatology Source Type: research