Martin Aringer, Daniel Toro-Domínguez, Marta E Alarcón-Riquelme
{"title":"Classification of systemic lupus erythematosus: From the development of classification criteria to a new taxonomy?","authors":"Martin Aringer, Daniel Toro-Domínguez, Marta E Alarcón-Riquelme","doi":"10.1016/j.berh.2024.101949","DOIUrl":"10.1016/j.berh.2024.101949","url":null,"abstract":"<p><p>SLE is a highly variable systemic autoimmune disease. Its immunopathological effector phase is partly understood. However, the background of its variability is not. SLE classification criteria have been relying on the clinical manifestations and standard autoimmune serology. This still holds true for the 2019 EULAR/ACR classification criteria. On one hand, this has led to significant precision in defining patients with SLE. On the other hand, the information in the criteria neither helps understanding the individual patient's pathophysiology, nor does it predict the efficacy of the available immunomodulatory therapies. Chances of further improvement of clinical criteria are most likely limited. This is where new multi-omic approaches have started to make an impact. While not yet able to differentiate diseases with the same precision as the classification criteria, the results of these studies go far beyond the scope of the criteria with regard to immune dysregulation. Looking at both sides in detail, we here try to synthesize the available data, aiming at a better understanding of SLE and its immune pathophysiology.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101949"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140904974","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Juan M Mejia-Vilet, Tabitha Turner-Stokes, Frederic Houssiau, Brad H Rovin
{"title":"Kidney involvement in systemic lupus erythematosus: From the patient assessment to a tailored treatment.","authors":"Juan M Mejia-Vilet, Tabitha Turner-Stokes, Frederic Houssiau, Brad H Rovin","doi":"10.1016/j.berh.2023.101925","DOIUrl":"10.1016/j.berh.2023.101925","url":null,"abstract":"<p><p>In the last few years, several studies have provided new evidence for the diagnosis, management, and follow-up of patients with lupus nephritis. Evidence showing dissociation between clinical and histological findings has prompted reevaluation of the role of the kidney biopsy as a tool for diagnosis and follow-up. In therapeutics, four immunosuppressive schemes now have supporting evidence for use as initial therapy. Current challenges include individualized selection of the best immunosuppressive regimen, an unmet need for non-invasive biomarkers of disease activity to inform treatment responses and guide subsequent therapy, holistic patient management in this complex, multisystem disease, and ultimately the development of more targeted therapies directed at specific effector pathways driving glomerular inflammation and damage in order to improve treatment response. In this communication, we review the diagnostic and therapeutic approach to lupus nephritis, as well as evaluation of response to therapy and disease control.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101925"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139049734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mariele Gatto, Roberto Depascale, Ana Luisa Stefanski, Eva Schrezenmeier, Thomas Dörner
{"title":"Translational implications of newly characterized pathogenic pathways in systemic lupus erythematosus.","authors":"Mariele Gatto, Roberto Depascale, Ana Luisa Stefanski, Eva Schrezenmeier, Thomas Dörner","doi":"10.1016/j.berh.2023.101864","DOIUrl":"10.1016/j.berh.2023.101864","url":null,"abstract":"<p><p>Improved characterization of relevant pathogenic pathways in systemic lupus erythematosus (SLE) has been further delineated over the last decades. This led to the development of targeted treatments including belimumab and anifrolumab, which recently became available in clinics. Therapeutic targets in SLE encompass interferon (IFN) signaling, B-T costimulation including immune checkpoints, and increasing modalities of B lineage targeting, such as chimeric antigen receptor (CAR) T cells directed against CD19 or sequential anti-B cell targeting. Patient profiling based on characterization of underlying molecular abnormalities, often performed through comprehensive omics analyses, has recently been shown to better predict patients' treatment responses and also holds promise to unravel key molecular mechanisms driving SLE. SLE carries two key signatures, namely the IFN and B lineage/plasma cell signatures. Recent advances in SLE treatments clearly indicate that targeting innate and adaptive immunity is successful in such a complex autoimmune disease. Although those signatures may interact at the molecular level and provide the basis for the first selective treatments in SLE, it remains to be clarified whether these distinct treatments show different treatment responses among certain patient subsets. In fact, notwithstanding the remarkable amount of novel clues for innovative SLE treatment, harmonization of big data within tailored treatment strategies will be instrumental to better understand and treat this challenging autoimmune disorder. This review will provide an overview of recent improvements in SLE pathogenesis, related insights by analyses of big data and machine learning as well as technical improvements in conducting clinical trials with the ultimate goal that translational research results in improved patient outcomes.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101864"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10448758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Elena Elefante, Alain Cornet, Jeanette Andersen, Amy Somers, Marta Mosca
{"title":"The communication GAP between patients and clinicians and the importance of patient reported outcomes in Systemic Lupus Erythematosus.","authors":"Elena Elefante, Alain Cornet, Jeanette Andersen, Amy Somers, Marta Mosca","doi":"10.1016/j.berh.2024.101939","DOIUrl":"10.1016/j.berh.2024.101939","url":null,"abstract":"<p><p>Systemic Lupus Erythematosus (SLE) imposes a great burden on the lives of patients. Patients' and physicians' concerns about the disease diverge considerably. Physicians focus on controlling disease activity to prevent damage accrual, while patients focus on symptoms that impact on Health-Related Quality of Life (HRQoL). We explored the physicians' and patients' perspective and the potential role of Patient Reported Outcomes (PROs). Physicians are aware of the theoretical usefulness of PROs to collect information deriving from the patients' perspective. However, they often do not know how to interpret and use these questionnaires in a real shared therapeutic strategy. For the patients, it's important to be seen as a whole person with a true consideration of how they feel and function. Strategies to help bridge the communication gap could include: better use of time during visits, preparing for the consultation, a more understandable lay language used by the doctor, a dedicated nurse.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101939"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140133186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pregnancy in systemic lupus erythematosus.","authors":"Dina Zucchi, Rebecca Fischer-Betz, Chiara Tani","doi":"10.1016/j.berh.2023.101860","DOIUrl":"10.1016/j.berh.2023.101860","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which frequently affects women of childbearing age. Nowadays, pregnancy is not contraindicated in cases of well-controlled disease activity, but pregnancies are still at higher risk of maternal and fetal complications compared to the general population. During pregnancy and puerperium patients are at risk of disease flare, and obstetric complications are more frequent in cases of active disease at conception/beginning of pregnancy, a history of lupus nephritis, and concomitant presence of antiphospholipid syndrome. To improve pregnancy outcomes in SLE patients, appropriate preconception counseling with changes in medication, if necessary, and close rheumatological and obstetrical monitoring are fundamental. This review aims to summarize the risk factors for adverse pregnancy outcomes and provide an update on developments in medical care for pregnancy in SLE patients.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101860"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10382136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Systemic lupus erythematosus and glucocorticoids: A never-ending story?","authors":"Diana Paredes-Ruiz, Guillermo Ruiz-Irastorza, Zahir Amoura","doi":"10.1016/j.berh.2023.101873","DOIUrl":"10.1016/j.berh.2023.101873","url":null,"abstract":"<p><p>Glucocorticoids (GCs) continue to be essential agents for the management of systemic lupus erythematosus, since there are no other drugs able to active remission of active disease so rapidly. However, their potential for causing irreversible damage greatly limit their use. Fortunately, some strategies may help take advantage of their huge anti-inflammatory power while limiting GC-induced side effects. This article reviews the pharmacological basis of GC action and their translation into the clinical ground. We also offer the practical approach for the use of GC in induction and maintenance therapy as well as the strategies for GC withdrawal of the respective practice of the authors. The three main basic principles are a) using methyl-prednisolone pulses to induce remission not only in severe disease; b) limiting initial doses of prednisone to ≤30 mg/d, with rapid tapering to ≤5 mg/d, which should be the dose for maintenance therapy; and c) individualizing the decision and the strategy to withdraw GCs. Long-term therapy with HCQ and the early introduction of immunosuppressive treatment would help achieve these objectives.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"101873"},"PeriodicalIF":4.5,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"92157242","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kelly Di Dier , Dominik Deppe , Torsten Diekhoff , Nele Herregods , Lennart Jans
{"title":"Clash of the titans: Current CT and CT-like imaging modalities in sacroiliitis in spondyloarthritis","authors":"Kelly Di Dier , Dominik Deppe , Torsten Diekhoff , Nele Herregods , Lennart Jans","doi":"10.1016/j.berh.2023.101876","DOIUrl":"10.1016/j.berh.2023.101876","url":null,"abstract":"<div><p><span>Sacroiliitis is characterised by active and structural changes of the joint. While the Assessment of </span>Spondyloarthritis<span><span> international Society (ASAS) classification criteria stress the importance of bone marrow inflammation, recent reports suggest that osteitis can occur in various diseases, mechanical conditions and healthy individuals. Thus, structural lesions such as joint surface erosion and </span>ankylosis<span> are important factors for differential diagnosis. Various imaging modalities are available to examine these changes. However, computed tomography (CT) is generally considered the reference standard. Nonetheless, recent advances in magnetic resonance imaging (MRI) allow for direct bone imaging and the reconstruction of CT-like images that can provide similar information. This way, the ability of MRI to detect and measure structural lesions is strengthened. The aim of this review is to provide an overview of the pros and cons of CT and CT-like imaging modalities in sacroiliitis.</span></span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"37 3","pages":"Article 101876"},"PeriodicalIF":5.2,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89720450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
María Ángeles Puche-Larrubia , Clementina López-Medina , Nelly Ziadé
{"title":"Peripheral spondyloarthritis: What have we learned?","authors":"María Ángeles Puche-Larrubia , Clementina López-Medina , Nelly Ziadé","doi":"10.1016/j.berh.2023.101862","DOIUrl":"10.1016/j.berh.2023.101862","url":null,"abstract":"<div><p>The peripheral spondyloarthritis<span> (pSpA) entity remains poorly defined in comparison with axial SpA and psoriatic arthritis, as the clinical symptoms have low specificity, the biological markers are virtually lacking, and dedicated randomized controlled trials<span> in this specific indication remain scarce. In addition, clinical similarities between pSpA and psoriatic arthritis (PsA) have been described, partly explained by a resemblance in the pathophysiology of both entities. Thus, diagnosing pSpA can be challenging because of the overlap with other entities and the absence of a specific test or imaging study that can definitively diagnose the condition.</span></span></p><p>The aim of this review is to summarize the current understanding of pSpA, its epidemiology, physiopathology, clinical diagnosis, and classification criteria. In addition, we present patient-reported outcomes used in pSpA clinical studies, available evidence on therapies, and future directions.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"37 3","pages":"Article 101862"},"PeriodicalIF":5.2,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10031950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Samantha O. Kohn , Abeera Azam , Lauren E. Hamilton , Stephanie R. Harrison , Elizabeth R. Graef , Kristen J. Young , Helena Marzo-Ortega , Jean W. Liew
{"title":"Impact of sex and gender on axSpA diagnosis and outcomes","authors":"Samantha O. Kohn , Abeera Azam , Lauren E. Hamilton , Stephanie R. Harrison , Elizabeth R. Graef , Kristen J. Young , Helena Marzo-Ortega , Jean W. Liew","doi":"10.1016/j.berh.2023.101875","DOIUrl":"10.1016/j.berh.2023.101875","url":null,"abstract":"<div><p><span>Axial spondyloarthritis<span> (axSpA) was historically considered a disease of men, largely due to the recognition of a more severe, progressive phenotype, ankylosing spondylitis (AS; or radiographic axSpA, r-axSpA) aiding the clinical diagnosis [</span></span><span>[1]</span>, <span>[2]</span><span>]. Data demonstrating the near equal prevalence of axSpA in women only started to emerge in the last decades, highlighting intrinsic differences in disease phenotype, and clinical and imaging characteristics between sexes, which partly explain the issue of underdiagnosis in women. Similar to the evolving understanding of spondyloarthritis and the diseases that term describes, the concepts of gender and sex<span> also warrant further clarification to accurately assess their potential role in disease pathophysiology and phenotypic expression. This narrative review delves into the most recent evidence from the literature on the true prevalence of sex differences in axSpA, and the impact of sex and gender on diagnosis, disease characteristics and treatment response in this, still underserved, chronic disease.</span></span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"37 3","pages":"Article 101875"},"PeriodicalIF":5.2,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138441657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pathophysiology and immunolgical basis of axial spondyloarthritis","authors":"Marleen G.H. van de Sande , Dirk Elewaut","doi":"10.1016/j.berh.2023.101897","DOIUrl":"10.1016/j.berh.2023.101897","url":null,"abstract":"<div><p>Over the recent years the wider availability and application of state-of-the-art immunological technologies greatly advanced the insight into the mechanisms that play an important role in axial spondyloarthritis<span> (axSpA) pathophysiology<span>. This increased understanding has facilitated the development of novel treatments that target disease relevant pathways, hereby improving outcome for axSpA patients.</span></span></p><p>In axSpA pathophysiology genetic and environmental factors as well as immune activation by mechanical or bacterial stress resulting in a chronic inflammatory response have a central role. The TNF and IL-23/IL-17 immune pathways play a pivotal role in these disease mechanisms.</p><p><span>This review provides an outline of the immunological basis of axSpA with a focus on key genetic risk factors and their link to activation of the pathological immune response, as well as on the role of the gut and </span>entheses in the initiation of inflammation with subsequent new bone formation in axSpA.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"37 3","pages":"Article 101897"},"PeriodicalIF":5.2,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138464126","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}