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Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus
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Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Immunology".

Deadline for manuscript submissions: closed (25 December 2023) | Viewed by 10380

Special Issue Editors

Prof. Dr. Christopher Sjöwall

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Guest Editor
Department of Biomedical and Clinical Sciences, Division of Inflammation and Infection/Rheumatology, Linköping University, SE-581 85 Linköping, Sweden
Interests: systemic lupus erythematosus; lupus nephritis; autoantibodies; biomarkers; type I interferons; pentraxins; complement; epidemiology
Special Issues, Collections and Topics in MDPI journals
Dr. Ioannis Parodis

E-Mail Website
Guest Editor
Department of Medicine Solna, Division of Rheumatology, Karolinska Institutet and Karolinska University Hospital, SE-171 76 Stockholm, Sweden
Interests: systemic lupus erythematosus; lupus nephritis; autoantibodies; biomarkers; B cells; outcome measures; treatment; biological therapies; health-related quality of life; patient-reported outcomes
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

In August 2020, the first volume of the Special Issue “Clinical Features and Long-Term Outcomes of Systemic Lupus Erythematosus” was launched (https://www.mdpi.com/journal/jcm/special_issues/lupus_erythematosus). We received many excellent papers on different aspects of systemic lupus erythematosus (SLE). Now, we would like to launch the second volume on this topic.

The clinical spectrum of SLE is highly heterogeneous, ranging from mild disease, which can be limited to skin and joint involvement, to life-threatening conditions with renal impairment, severe cytopenias, central nervous system disease, and thromboembolic events. Apart from the host genetics, several environmental factors, such as sunlight, infections, drugs, and probably hormonal factors, can trigger the onset of symptoms related to SLE. Despite significant advances in our understanding of the pathophysiology and optimization of medical care, patients with SLE still have significant premature mortality and many patients experience severe disease with increased risk to acquire organ damage and reduced health-related quality of life. Development of effective drugs that can induce remission or low disease activity, unanimous use of definitions of remission and low or high disease activity, flare, and response to therapy, identification of non-invasive biomarkers of disease activity and long-term outcomes, and implementation of SLE patients’ perspective as an integral part of the clinical assessment constitute only a few of the many unmet needs in the field of SLE.

This Special Issue aims to provide a broad overview of current and new knowledge on clinical features and long-term outcomes of SLE, including disease phenotypes, therapeutic strategies, and biomarkers. Research incorporating the COVID-19 viral pandemic and its consequences for SLE patients is also welcome.

Yours faithfully

Prof. Dr. Christopher Sjöwall
Dr. Ioannis Parodis
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • systemic lupus erythematosus
  • lupus nephritis
  • autoantibodies
  • biomarkers
  • clinical follow-up
  • epidemiology
  • outcome measures
  • treatment
  • health-related quality of life
  • patient-reported outcomes

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Related Special Issue

Published Papers (4 papers)

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Research

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13 pages, 6380 KiB  
Article
Progressive Multifocal Leukoencephalopathy in Systemic Lupus Erythematosus: A Consequence of Patient-Intrinsic or -Extrinsic Factors?
by Evgenia Emmanouilidou, Despoina Kosmara, Efrosini Papadaki, Vasileios Mastorodemos, Pantelis Constantoulakis, Argyro Repa, Georgia Christopoulou, Christina Kalpadakis, Nestor Avgoustidis, Konstantinos Thomas, Dimitrios Boumpas, Prodromos Sidiropoulos and George Bertsias
J. Clin. Med. 2023, 12(21), 6945; https://doi.org/10.3390/jcm12216945 - 6 Nov 2023
Cited by 3 | Viewed by 2089
Abstract
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS) caused by reactivation of the polyomavirus JC (JCV) typically in immunocompromised individuals. The risk of PML among rheumatic diseases may be higher for systemic lupus erythematosus (SLE), without, [...] Read more.
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS) caused by reactivation of the polyomavirus JC (JCV) typically in immunocompromised individuals. The risk of PML among rheumatic diseases may be higher for systemic lupus erythematosus (SLE), without, however, a clear association with the type and intensity of background therapy. We present the development and outcome of PML in a 32-year-old female lupus patient under mild immunosuppressive treatment, yet with marked B-cell lymphopenia in the peripheral blood and bone marrow (<1% of total lymphocytes). Despite treatment with the immune checkpoint inhibitor pembrolizumab, the patient showed progressive neurological and brain imaging deterioration and eventually died 15 months after PML diagnosis. To unveil possible underlying genetic liabilities, whole exome sequencing was performed which identified deleterious variants in GATA2 and CDH7 genes, which both have been linked to defective T- and/or B-lymphocyte production. These findings reiterate the possible role of disease-/patient-intrinsic factors, rather than that of drug-induced immunosuppression, in driving immune dysregulation and susceptibility to PML in certain patients with SLE. Full article
(This article belongs to the Special Issue Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus)
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Review

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15 pages, 948 KiB  
Review
Therapeutic Drug Monitoring in Patients with Systemic Lupus Erythematosus: Utility and Gaps
by Kar Mun Chong, He Jiang, Elaine Ah Gi Lo, Wei-Zhen Hong, Emmett Tsz-Yeung Wong, Gek Cher Chan and Jiacai Cho
J. Clin. Med. 2024, 13(2), 451; https://doi.org/10.3390/jcm13020451 - 13 Jan 2024
Viewed by 2206
Abstract
Despite advances in the treatment of patients with systemic lupus erythematous (SLE), outcomes have remained suboptimal. Persistent disease activity, patient comorbidities and drug toxicities contribute to the accrual of progressive irreversible damage and high rates of morbidity and mortality. Currently, similar drug doses [...] Read more.
Despite advances in the treatment of patients with systemic lupus erythematous (SLE), outcomes have remained suboptimal. Persistent disease activity, patient comorbidities and drug toxicities contribute to the accrual of progressive irreversible damage and high rates of morbidity and mortality. Currently, similar drug doses and regimens are promulgated in the treatment guidelines for all SLE patients, despite the vast differences in patient and environmental factors that affect the drugs’ metabolism and blood concentrations. This causes a disconnect between drug dosing and drug blood concentrations, which can then result in unpredictability in drug toxicities and therapeutic effects. In this review, we discuss commonly used oral immunosuppressive medications in SLE, their pharmacogenomics, and factors affecting their metabolism and blood concentrations. Further, we highlight the role of therapeutic drug monitoring in SLE, which is the first accessible step to individualising therapy. Full article
(This article belongs to the Special Issue Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus)
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19 pages, 722 KiB  
Review
B Cell Tolerance and Targeted Therapies in SLE
by Ioannis Parodis, Xuan Long, Mikael C. I. Karlsson and Xin Huang
J. Clin. Med. 2023, 12(19), 6268; https://doi.org/10.3390/jcm12196268 - 28 Sep 2023
Cited by 4 | Viewed by 3410
Abstract
Systemic Lupus Erythematosus (SLE) is a chronic systemic autoimmune disease of high clinical and molecular heterogeneity, and a relapsing-remitting pattern. The disease is currently without cure and more prevalent in women. B cell tolerance and production of autoantibodies are critical mechanisms that drive [...] Read more.
Systemic Lupus Erythematosus (SLE) is a chronic systemic autoimmune disease of high clinical and molecular heterogeneity, and a relapsing-remitting pattern. The disease is currently without cure and more prevalent in women. B cell tolerance and production of autoantibodies are critical mechanisms that drive SLE pathophysiology. However, how the balance of the immune system is broken and how the innate and adaptive immune systems are interacting during lupus-specific autoimmune responses are still largely unknown. Here, we review the latest knowledge on B cell development, maturation, and central versus peripheral tolerance in connection to SLE and treatment options. We also discuss the regulation of B cells by conventional T cells, granulocytes, and unconventional T cells, and how effector B cells exert their functions in SLE. We also discuss mechanisms of action of B cell-targeted therapies, as well as possible future directions based on current knowledge of B cell biology. Full article
(This article belongs to the Special Issue Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus)
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Other

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13 pages, 1531 KiB  
Systematic Review
Optimizing Patient Care: A Systematic Review of Multidisciplinary Approaches for SLE Management
by Giorgio Galoppini, Antonio Marangoni, Francesca Cirilli, Francesca Ruffilli, Carlo Garaffoni, Marcello Govoni, Carlo Alberto Scirè, Ettore Silvagni and Alessandra Bortoluzzi
J. Clin. Med. 2023, 12(12), 4059; https://doi.org/10.3390/jcm12124059 - 15 Jun 2023
Cited by 4 | Viewed by 1966
Abstract
Systemic lupus erythematosus (SLE) is characterized by multisystemic clinical manifestations ranging from a relatively mild involvement to potentially life-threatening complications. Due to this complexity, a multidisciplinary (MD) approach is the best strategy for optimizing patients’ care. The main aim of this systematic literature [...] Read more.
Systemic lupus erythematosus (SLE) is characterized by multisystemic clinical manifestations ranging from a relatively mild involvement to potentially life-threatening complications. Due to this complexity, a multidisciplinary (MD) approach is the best strategy for optimizing patients’ care. The main aim of this systematic literature review (SLR) was to scrutinize the published data regarding the MD approach for the management of SLE patients. The secondary objective was to evaluate the outcomes of the MD approach in SLE patients. The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. We performed an SLR to retrieve articles available in English or Italian listed in PubMed, Embase, Cinahl, and Cochrane Library concerning the MD approach used in observational studies and clinical trials. Four independent reviewers performed the study selection and data collection. Of 5451 abstracts evaluated, 19 studies were included in the SLR. The MD approach was most frequently described in the context of SLE pregnancy, reported in 10 papers. MD teams were composed of a rheumatologist, except for one cohort study; a gynecologist; a psychologist; a nurse; and other health professionals. MD approaches had a positive impact on pregnancy-related complications and disease flares and improved SLE psychological impact. Although international recommendations advise an MD approach for managing SLE, our review highlighted the paucity of data supporting this strategy, with most of the available evidence on the management of SLE during pregnancy. Full article
(This article belongs to the Special Issue Clinical Aspects and Unmet Needs in Systemic Lupus Erythematosus)
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