Disease activity assessment in patients with psoriatic arthritis
Publication date: Available online 11 October 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Andreas Kerschbaumer, Josef S. Smolen, Daniel AletahaAbstractPsoriatic arthritis (PsA) is an inflammatory disease in patients with a personal or family history of psoriasis. While synovitis is the major hallmark, enthesitis, dactylitis, axial inflammation, and skin and nail involvement are variable in prevalence and severity. The assessment of disease activity is important to determine the treatment target (“Treat-to-Target”) as well as early response to therapy, because a prolonged ineffective...
Source: Best Practice and Research Clinical Rheumatology - October 11, 2018 Category: Rheumatology Source Type: research

Pediatric forms of vasculitis
Publication date: Available online 10 October 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Seza Özen, Hafize Emine Sönmez, Selcan DemirAbstractPrimary vasculitides that affect children are a challenging and complex group of disorders that may involve any system of the body and lead to significant morbidity and mortality. In recent years, there have been significant advances in the field of childhood vasculitides, including the development of classification criteria and outcome assessment. Although some forms of vasculitis occur in both children and adults, considerable differences exis...
Source: Best Practice and Research Clinical Rheumatology - October 10, 2018 Category: Rheumatology Source Type: research

Update on the epidemiology, risk factors, and disease outcomes of psoriatic arthritis
Publication date: Available online 5 October 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Dilek Solmaz, Lihi Eder, Sibel Zehra AydinAbstractPsoriatic arthritis (PsA) is a chronic inflammatory disease that affects different structures of the musculoskeletal system in addition to the skin and the nail. The complexity of the disease had been a barrier to understand the pathogenesis and define valid outcome tools; however, our understanding about the disease has considerably increased with time mainly because of the advances in imaging, new discoveries in genetics and underlying inflammatory pathway...
Source: Best Practice and Research Clinical Rheumatology - October 6, 2018 Category: Rheumatology Source Type: research

Investigations in systemic vasculitis – The role of imaging
Publication date: Available online 25 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Wolfgang A. Schmidt, Daniel BlockmansAbstractImaging plays an increasing role for confirming a suspected diagnosis of giant cell arteritis (GCA) or Takayasu arteritis (TAK). Ultrasound, magnetic resonance imaging (MRI), and computed tomography demonstrate a homogeneous, most commonly concentric, arterial wall thickening. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) displays increased FDG uptake of inflamed artery walls delineating increased metabolism. Ultrasound and MRI are recommende...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Thumb base osteoarthritis: A hand osteoarthritis subset requiring a distinct approach
Publication date: Available online 26 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Margreet Kloppenburg, Sjoerd van Beest, Féline P.B. KroonAbstractHand osteoarthritis (OA) is usually a polyarticular disease, preferentially affecting the thumb base (TB) and interphalangeal joints. TB OA alone is generally not addressed separately, but as a part of hand OA. Studies have shown that OA in the TB joints clusters together, as does OA in the interphalangeal joints, supporting it as a distinct subset. Further support for this view comes from a specific risk profile, influence on clinic...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Gut inflammation in spondyloarthritis
Publication date: Available online 27 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Aroldo Rizzo, Angelo Ferrante, Giuliana Guggino, Francesco CicciaAbstractSpondyloarthritis (SpA) is a group of related diseases sharing common etiopathogenic mechanisms and clinical manifestations supported by a complex genetic predisposition. Gut inflammation is present in patients with SpA including patients showing clinically evident intestinal inflammation in the form of Crohn's disease or ulcerative colitis and patients who despite the absence of signs and symptoms of intestinal inflammation display...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Update on the epidemiology, risk factors, and disease outcomes of systemic lupus erythematosus
Publication date: Available online 27 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Irini Gergianaki, Alessandra Bortoluzzi, George BertsiasAbstractObtaining an updated view of the epidemiology, risk factors, and prognosis of systemic lupus erythematosus (SLE) is pivotal to our understanding of the disease burden. Recent community-based studies with comprehensive methodology provided more accurate disease occurrence estimates and suggested that SLE may be more frequent than previously thought. Gender, race, and socioeconomic status are important disease determinants, and there is increa...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Update on the epidemiology and disease outcome of Familial Mediterranean fever
Publication date: Available online 29 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Seza ÖzenAbstractAutoinflammatory diseases (AIDs) are diseases of the innate immune system, with clinical and laboratory evidence of attacks of inflammation. The more common AIDs are those associated with periodic fevers: Familial Mediterranean fever (FMF); Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS); Cryopyrin-associated Autoinflammatory Syndrome (CAPS); and Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS). Classification criteria have been developed ...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Genetics in ankylosing spondylitis – Current state of the art and translation into clinical outcomes
Publication date: Available online 3 October 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Matthew A. Brown, B. Paul WordsworthAbstractAnkylosing spondylitis (AS) is the prototypic form of axial spondyloarthritis (axSpA). It is highly heritable, with studies conducted in twins and in unrelated cases and controls showing that the heritability for AS is much higher than those for inflammatory bowel disease or rheumatoid arthritis. To date, 116 loci have been identified, contributing to 28% of the genetic variation in the disease. These loci provide important clues into pathogenic pathways in the di...
Source: Best Practice and Research Clinical Rheumatology - October 5, 2018 Category: Rheumatology Source Type: research

Update on the epidemiology, risk factors and disease outcomes of Behçet's disease
Publication date: Available online 21 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Nurullah AkkoçAbstractBehçet's disease (BD) may be regarded as a polygenic autoinflammatory disease although adaptive immune system has also been implicated in pathogenesis. Different classification criteria sets exist for BD, including the new “International Criteria for BD.” The pooled prevalence of BD was calculated as 10.3 per 100,000 population globally. BD is common along the Silk Road, including Turkey. Male sex and early onset are associated with a more severe disease co...
Source: Best Practice and Research Clinical Rheumatology - September 22, 2018 Category: Rheumatology Source Type: research

Prognosis and future developments in vasculitis
Publication date: Available online 22 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): N. Basu, M. Karabayas, C. PuseyAbstractThe prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of invariably acute and fatal conditions, but these disorders are currently considered to be chronic diseases. This change is largely attributable to earlier diagnosis and the careful application of immunotherapeutics. However, patients still experience premature mortality, relapse, comorbid ill health and poor quality of life. Mortality rates in large-vessel vasculit...
Source: Best Practice and Research Clinical Rheumatology - September 22, 2018 Category: Rheumatology Source Type: research

Oral treatment options for AS and PsA: DMARDs and small-molecule inhibitors
Publication date: Available online 20 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Jonathan Chan, Dafna GladmanAbstractSpondyloarthritis (SpA) represents a group of common diseases that share a number of characteristic clinical manifestations including peripheral arthritis, spondylitis, enthesitis, and dactylitis. Additionally, they can often be associated with extra-articular manifestations including psoriasis, anterior uveitis, and inflammatory bowel disease. The two most widely studied clinical phenotypes are ankylosing spondylitis and psoriatic arthritis.Although a number of biolog...
Source: Best Practice and Research Clinical Rheumatology - September 20, 2018 Category: Rheumatology Source Type: research

Evolving concepts of new bone formation in axial spondyloarthritis: Insights from animal models and human studies
Publication date: Available online 20 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Rik J. Lories, Nigil HaroonAbstractNew bone formation potentially leading to ankylosis of the sacroiliac joints and the spine is the main type of structural damage to the skeleton that characterizes axial spondyloarthritis. New data from animal models, imaging, and patient cohort studies support the view that sustained suppression of inflammation by therapeutic interventions can slow down the ankylosing process, at least in a large proportion of the patients. Although specific growth factor molecular sig...
Source: Best Practice and Research Clinical Rheumatology - September 20, 2018 Category: Rheumatology Source Type: research

The role of atherosclerosis in osteoarthritis
Publication date: Available online 14 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): S.M.A. Bierma-Zeinstra, J.H. WaarsingAbstractIn recent years, several papers that report an association between atherosclerosis (AS) and osteoarthritis (OA) have been published. Studying this relation is, however, not a sinecure owing to the multifactorial nature of these diseases and their long lead-in times. In this review, we discuss the evidence that is currently available for the association between OA and both subclinical AS and AS-related cardiovascular disease (CVD) events. We found that evidence...
Source: Best Practice and Research Clinical Rheumatology - September 15, 2018 Category: Rheumatology Source Type: research

Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis
Publication date: Available online 14 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Francesca Ingegnoli, Nicola Ughi, Carina MihaiAbstractSystemic sclerosis (SSc) is a chronic immune-mediated connective tissue disease with heterogeneous organ involvement. New classification criteria were developed allowing disease identification even before the onset of its hallmark, skin fibrosis. Incidence and prevalence vary among reports and are influenced by methodology. Despite earlier diagnosis, mortality of SSc is still considerable, mainly because of cardiopulmonary causes. Genetic predispositi...
Source: Best Practice and Research Clinical Rheumatology - September 14, 2018 Category: Rheumatology Source Type: research

Soluble biochemical markers of osteoarthritis: Are we close to using them in clinical practice?
Publication date: Available online 11 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Ali Mobasheri, Anne-Christine Bay-Jensen, Oreste Gualillo, Jonanthan Larkin, Marc C. Levesque, Yves HenrotinAbstractOsteoarthritis (OA) is the most common form of arthritis and a major cause of pain and disability. Recent work suggests that the global burden of OA is increasing, and costs associated with treatment are expected to increase dramatically as the aging human population expands. OA is currently diagnosed using radiography, but this technique is an indirect and insensitive measure of alteration...
Source: Best Practice and Research Clinical Rheumatology - September 12, 2018 Category: Rheumatology Source Type: research

Osteoarthritis: Research in motion
Publication date: Available online 7 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Francis Berenbaum, Margreet Kloppenburg (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - September 8, 2018 Category: Rheumatology Source Type: research

Preface: Future trends in the field of spondyloarthritis
Publication date: Available online 6 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Jonathan Sherlock, Peter C. Taylor (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - September 7, 2018 Category: Rheumatology Source Type: research

Born to run: The paradox of biomechanical force in spondyloarthritis from an evolutionary perspective
Publication date: Available online 1 September 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Karlijn Debusschere, Isabelle Cambré, Eric Gracey, Dirk ElewautAbstractSpondyloarthritis (SpA) refers to a group of chronic inflammatory arthritic diseases that can be severely debilitating. The most common form of SpA affecting the peripheral skeleton is psoriatic arthritis, while that affecting the axial skeleton is ankylosing spondylitis. SpA has a multifactorial nature, with both genetic and environmental factors initiating and maintaining the disease. Recently, the role of biomechanical stress...
Source: Best Practice and Research Clinical Rheumatology - September 1, 2018 Category: Rheumatology Source Type: research

Th17 cell responses in spondyloarthritis
Publication date: Available online 30 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): J.S.H. Gaston, D.R. JadonAbstractTargeting IL-17 has become an important option in the current treatment of spondyloarthritis (SpA). To place this therapeutic advancement in context, we review the discovery and properties of this cytokine, noting those which predispose to inflammation and led to it being considered as an attractive target for the treatment of arthritis, especially SpA. The processes that regulate the differentiation of IL-17-producing cells, particularly Th17 CD4+ T cells, have been investi...
Source: Best Practice and Research Clinical Rheumatology - August 31, 2018 Category: Rheumatology Source Type: research

The complications of vasculitis and its treatment
Publication date: Available online 28 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Catherine King, Lorraine Harper, Mark LittleAbstractSurvival following a diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has improved since the introduction of cyclophosphamide-based immunosuppressive regimens and is now almost 80% at 5 years. However, mortality remains 2.6 times greater in the population with AAV than in an age- and sex-matched general population. The greatest risk of harm for patients with AAV is during the first year of diagnosis and from the adverse ...
Source: Best Practice and Research Clinical Rheumatology - August 29, 2018 Category: Rheumatology Source Type: research

Conventional and biological immunosuppressants in vasculitis
Publication date: Available online 22 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Loic Guillevin, Chetan Mukhtyar, Christian Pagnoux, Max YatesAbstractThe following chapter outlines the main findings from clinical trials, which provide information on the current best evidence-based management of the myriad of conditions that comprise vasculitis.Glucocorticoids (GCs) have been the mainstay of treatment of large-vessel vasculitis since Birkhead et al. used intramuscular cortisone daily and obtained good results in patients with giant cell arteritis. Recent trial data offer the hope th...
Source: Best Practice and Research Clinical Rheumatology - August 23, 2018 Category: Rheumatology Source Type: research

The role of HLA-B*27 in spondyloarthritis
Publication date: Available online 23 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Robert A. Colbert, Fatemeh Navid, Tejpal GillAbstractThe mechanism by which HLA-B*27 predisposes to spondyloarthritis remains unresolved. Arthritogenic peptides have not been defined in humans and are not involved in experimental models of spondyloarthritis. Aberrant properties of HLA-B*27 can activate the IL-23/IL-17 axis in HLA-B*27 transgenic rats and humans. In HLA-B*27-independent rodent models, spondyloarthritis can be driven by IL-23 triggering entheseal-resident CD4-/CD8- T cells or CD4+ Th17 T...
Source: Best Practice and Research Clinical Rheumatology - August 23, 2018 Category: Rheumatology Source Type: research

Pain in spondyloarthritis: A neuro–immune interaction
Publication date: Available online 17 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Ejaz M.I. Pathan, Robert D. InmanAbstractPain in spondyloarthritis is usually attributed to inflammation. However, treatment with potent biologic agents that control inflammation does not always control the pain. Pain is hence likely to be multifactorial. Fatigue is another prominent feature of this condition which again tends to respond poorly to potent biologic agents. There is also a growing interest in coexisting fibromyalgia in this group of patients and how this affects response to biologic therapies....
Source: Best Practice and Research Clinical Rheumatology - August 17, 2018 Category: Rheumatology Source Type: research

Surgical intervention and its role in Takayasu arteritis
Publication date: Available online 17 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Justin C. MasonAbstractVascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ ischaemia or life-threatening aneurysm formation. In most cases, this follows the failure of medical therapy to prevent arterial injury. Open surgery and endovascular approaches are used. The choice between them, at least in part, is determined by the site and nature of the lesion. Open surgery, although more invasive, ...
Source: Best Practice and Research Clinical Rheumatology - August 17, 2018 Category: Rheumatology Source Type: research

Investigations in systemic vasculitis. The role of the laboratory
Publication date: Available online 16 August 2018Source: Best Practice & Research Clinical RheumatologyAuthor(s): Elena Csernok, Xavier BossuytAbstractThe diagnosis of systemic vasculitis is challenging. Laboratory testing may provide useful information. Routine laboratory tests include erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulin, cryoglobulin, and Hepatitis B and C serology. Although ESR and CRP are often helpful for the diagnosis of vasculitis, they are nonspecific and do not help in distinguishing betwe...
Source: Best Practice and Research Clinical Rheumatology - August 16, 2018 Category: Rheumatology Source Type: research

The SKG model of spondyloarthritis
We describe the contribution of animal models to the earliest known disease mechanisms, including how attenuated T cell signalling contributes to disease pathogenesis as a result of genetic or environmental predisposition. We also discuss recent advances in the understanding of key cytokines that drive SpA pathophysiology and the crosstalk of gut microbiota with host genetic determinants of immune function and finally the potential future prospects to study these animal models to translate our knowledge of pathogenesis into clinical practice. (Source: Best Practice and Research Clinical Rheumatology)
Source: Best Practice and Research Clinical Rheumatology - August 8, 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 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