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Biomolecules Jun 2021Systemic lupus erythematosus (SLE) is characterized by immune system dysfunction and is clinically heterogeneous, exhibiting renal, dermatological, neuropsychiatric, and... (Review)
Review
Systemic lupus erythematosus (SLE) is characterized by immune system dysfunction and is clinically heterogeneous, exhibiting renal, dermatological, neuropsychiatric, and cardiovascular symptoms. Clinical and physiological assessment is usually inadequate for diagnosing and assessing pathophysiological processes in SLE. Clinical and immunological biomarkers could play a critical role in improving diagnosis, assessment, and ultimately, control of SLE. This article reviews clinical and immunological biomarkers that could diagnose and monitor disease activity in SLE, with and without organ-specific injury. In addition, novel SLE biomarkers that have been discovered through "omics" research are also reviewed.
Topics: Biomarkers; Humans; Lupus Erythematosus, Systemic
PubMed: 34206696
DOI: 10.3390/biom11070928 -
Clinical and Experimental Rheumatology Jan 2022Systemic lupus erythematosus (SLE) is a chronic multisystem auto-immune disease with extremely varied clinical manifestations and a complex pathogenesis. New insights in... (Review)
Review
Systemic lupus erythematosus (SLE) is a chronic multisystem auto-immune disease with extremely varied clinical manifestations and a complex pathogenesis. New insights in SLE about pathogenetic pathways, biomarkers, and data on clinical manifestations are progressively emerging, and new drugs and new therapeutic strategies have been proposed to improve the control of disease activity. Thus, this review is aimed to summarise the most relevant data about SLE emerged during 2021, following the previous annual review of this series.
Topics: Biomarkers; Humans; Lupus Erythematosus, Systemic
PubMed: 35088691
DOI: 10.55563/clinexprheumatol/nolysy -
Annual Review of Medicine Jan 2023Systemic lupus erythematosus (SLE) is a devastating autoimmune disease that can result in substantial morbidity and mortality. Diagnosis and treatment of SLE are... (Review)
Review
Systemic lupus erythematosus (SLE) is a devastating autoimmune disease that can result in substantial morbidity and mortality. Diagnosis and treatment of SLE are clinical challenges. Patient presentation and response to therapy are heterogeneous because of the complex immune dysregulation that results in SLE disease pathogenesis. An intricate interplay between genetic risk and skewing of adaptive and innate immune system responses leads to overproduction of type I interferons and other cytokines, complement activation, immune-complex deposition, and ultimately inflammation and tissue damage. Here, we review the classification criteria as well as standard and emerging diagnostic tools available to identify patients with SLE. We then focus on medical management, including novel therapeutics, nonpharmacologic interventions, and comorbidity management.
Topics: Humans; Immunity, Innate; Cytokines; Interferon Type I; Lupus Erythematosus, Systemic
PubMed: 35804480
DOI: 10.1146/annurev-med-043021-032611 -
Clinical and Experimental Rheumatology May 2023Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. New data regarding... (Review)
Review
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. New data regarding pathogenic pathways, biomarkers and clinical manifestations of SLE are emerging, and new drugs and therapeutic protocols have been proposed to improve the control of disease activity. Furthermore, new insights into comorbidities and reproductive health in SLE patients are constantly emerging.This annual review aims to summarise the most relevant data on SLE that was published in 2022.
Topics: Humans; Lupus Erythematosus, Systemic; Autoimmune Diseases; Comorbidity; Biomarkers
PubMed: 37133502
DOI: 10.55563/clinexprheumatol/4uc7e8 -
Current Opinion in Rheumatology Nov 2020To compare the recently published European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for SLE with the Systemic... (Review)
Review
PURPOSE OF REVIEW
To compare the recently published European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for SLE with the Systemic Lupus International Collaborating Centers (SLICC) criteria and the earlier ACR criteria, focusing on their key concepts.
RECENT FINDINGS
Although the SLICC criteria introduced numbers of new criteria items, the new EULAR/ACR criteria added only noninfectious fever, based on an early SLE cohort study and an SLE patient survey, and condensed hematological, mucocutaneous and neurological items. Whereas the SLICC criteria maintained the overall structure familiar from the ACR criteria, the EULAR /ACR criteria use antinuclear antibodies (ANA) as an obligatory entry criterion, have weighted criteria and group these in domains. Where the SLICC criteria greatly increased sensitivity, losing some specificity, the EULAR/ACR criteria increased specificity again, for excellent classification criteria performance.
SUMMARY
Despite differences in structure and statistical performance, the EULAR/ACR and SLICC criteria agree on the importance of both immunological and clinical findings, on the high impact of lupus nephritis by histology, and on most clinical items.
Topics: Antibodies, Antinuclear; Cohort Studies; Female; Humans; Lupus Erythematosus, Systemic; Male; Rheumatology
PubMed: 32925250
DOI: 10.1097/BOR.0000000000000740 -
Current Rheumatology Reports Feb 2021Juvenile-onset systemic lupus erythematosus ((j)SLE) is an autoimmune/inflammatory disease that results in significant damage and disability. When compared to patients... (Review)
Review
PURPOSE OF REVIEW
Juvenile-onset systemic lupus erythematosus ((j)SLE) is an autoimmune/inflammatory disease that results in significant damage and disability. When compared to patients with disease onset in adulthood, jSLE patients exhibit increased disease activity, damage and require more aggressive treatments. This manuscript summarises age-specific pathogenic mechanisms and underscores the need for age group-specific research, classification and treatment.
RECENT FINDINGS
Genetic factors play a significant role in the pathophysiology of jSLE, as > 7% of patients develop disease as a result of single gene mutations. Remaining patients carry genetic variants that are necessary for disease development, but require additional factors. Increased 'genetic impact' likely contributes to earlier disease onset and more severe phenotypes. Epigenetic events have only recently started to be addressed in jSLE, and add to the list of pathogenic mechanisms that may serve as biomarkers and/or treatment targets. To allow meaningful and patient-oriented paediatric research, age-specific classification criteria and treatment targets require to be defined as currently available tools established for adult-onset SLE have limitations in the paediatric cohort. Significant progress has been made in understanding the pathophysiology of jSLE. Meaningful laboratory and clinical research can only be performed using age group-specific tools, classification criteria and treatment targets.
Topics: Adolescent; Age of Onset; Child; Humans; Lupus Erythematosus, Systemic; Phenotype
PubMed: 33569643
DOI: 10.1007/s11926-021-00985-0 -
International Journal of Molecular... Mar 2023Systemic lupus erythematosus (SLE) is a genetically predisposed, female-predominant disease, characterized by multiple organ damage, that in its most severe forms can be... (Review)
Review
Systemic lupus erythematosus (SLE) is a genetically predisposed, female-predominant disease, characterized by multiple organ damage, that in its most severe forms can be life-threatening. The pathogenesis of SLE is complex and involves cells of both innate and adaptive immunity. The distinguishing feature of SLE is the production of autoantibodies, with the formation of immune complexes that precipitate at the vascular level, causing organ damage. Although progress in understanding the pathogenesis of SLE has been slower than in other rheumatic diseases, new knowledge has recently led to the development of effective targeted therapies, that hold out hope for personalized therapy. However, the new drugs available to date are still an adjunct to conventional therapy, which is known to be toxic in the short and long term. The purpose of this review is to summarize recent advances in understanding the pathogenesis of the disease and discuss the results obtained from the use of new targeted drugs, with a look at future therapies that may be used in the absence of the current standard of care or may even cure this serious systemic autoimmune disease.
Topics: Female; Humans; Adaptive Immunity; Autoantibodies; Antigen-Antibody Complex; Lupus Erythematosus, Systemic
PubMed: 37047548
DOI: 10.3390/ijms24076578 -
Rheumatology (Oxford, England) Dec 2020The EULAR/ACR 2019 classification criteria for SLE constitute a current and optimized clinical approach to SLE classification. Classification is still not based on... (Review)
Review
The EULAR/ACR 2019 classification criteria for SLE constitute a current and optimized clinical approach to SLE classification. Classification is still not based on molecular approaches and the results from large studies using polyomics may be interpreted as demonstrating the relevance of the genetic and environmental background rather than splitting SLE into several entities. In fact, an association study within the EULAR/ACR classification criteria project found associations between manifestations only within organ domains. This independency of various organ manifestations argues for SLE as one disease entity. The current review article will therefore concentrate on the clinical and immunological manifestations of SLE and on what we have already learned in this century. Moreover, the structure and essential rules of the EULAR/ACR 2019 classification criteria will be discussed. While classification and diagnosis are distinct concepts, which have to remain clearly separated, information derived from the process towards the classification criteria is also useful for diagnostic purposes. Therefore this article also tries to delineate what classification can teach us for diagnosis, covering a wide variety of SLE manifestations.
Topics: Antibodies, Antinuclear; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis; Musculoskeletal System; Skin
PubMed: 33280013
DOI: 10.1093/rheumatology/keaa379 -
Immunology Nov 2019Systemic lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease of unknown etiology, although genetic and environmental factors appear to contribute to... (Review)
Review
Systemic lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease of unknown etiology, although genetic and environmental factors appear to contribute to its pathogenesis. Specifically, infectious processes are associated with SLE onset and exacerbation. However, we are far from a complete understanding of the interactions between infectious agents and the host, explaining the interest in gathering updated scientific information on this topic. According to the literature, the pathogens most frequently associated with SLE are viruses, notably human endogenous retroviruses, Epstein-Barr virus, parvovirus B19, cytomegalovirus and human immunodeficiency virus type 1, alongside certain bacterial components that can also trigger activation of the immune system. The mechanisms underlying autoreactivity remain unclear but various explanations have been proposed, including immunological changes responsible for infectious processes or molecular mimicry between host structures and those of infectious agents.
Topics: Humans; Lupus Erythematosus, Systemic; Molecular Mimicry; Virus Diseases; Viruses
PubMed: 31386190
DOI: 10.1111/imm.13103 -
Current Rheumatology Reports Feb 2021Complement activation is a key event in the pathogenesis of tissue inflammation and injury in systemic lupus erythematosus (SLE). This review is aimed at comparing the... (Review)
Review
PURPOSE OF REVIEW
Complement activation is a key event in the pathogenesis of tissue inflammation and injury in systemic lupus erythematosus (SLE). This review is aimed at comparing the usefulness of measurement of complement proteins in serum/plasma (C3, C4) to complement activation (split) products in plasma and on circulating blood cells for SLE diagnosis, disease monitoring, and prognosis.
RECENT FINDINGS
Complement split products, C3dg, iC3b, and C4d, are elevated in SLE, and C3dg/C3 and iC3b/C3 ratios correlate with active SLE. C4d also is higher in patients with lupus nephritis. An elevated level of the alternative pathway split product, Bb, in early lupus pregnancy is a predictor of adverse outcomes in SLE patients with antiphospholipid antibodies. Elevated levels of cell-bound complement activation products (CB-CAPs), namely, B cell-bound C4d (BC4d) and erythrocyte-bound C4d (EC4d), within a multiparameter assay panel, may predict transition to SLE more than other lupus biomarkers. EC4d better correlates with lupus disease activity than low plasma complement levels. Elevated platelet-bound C4d (PC4d) correlates with thrombosis in SLE. Both EC4d and PC4d are increased in primary and secondary anti-phospholipid syndrome, and anti-beta2glycoproteinI antibodies may directly activate the complement system. Abnormal levels of plasma complement split products and CB-CAPs support complement activation as an important pathogenetic mechanism in SLE and the antiphospholipid syndromes. These tests show promise for the diagnosis of SLE and monitoring of disease activity.
Topics: Antiphospholipid Syndrome; Biomarkers; Complement Activation; Complement System Proteins; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis
PubMed: 33569681
DOI: 10.1007/s11926-021-00984-1