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Pediatric Clinics of North America Apr 2012Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of disease manifestations that can involve any organ system, and can lead to... (Review)
Review
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of disease manifestations that can involve any organ system, and can lead to significant morbidity and even mortality. This article reviews the epidemiology, common clinical features, and complications of the disease, and briefly discusses the available treatment options. In addition, important medical and psychosocial issues relevant to the pediatrician caring for children and adolescents with SLE are discussed.
Topics: Adolescent; Child; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Treatment Outcome
PubMed: 22560574
DOI: 10.1016/j.pcl.2012.03.007 -
The Journal of Rheumatology Jan 2023The majority of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations at some point in their disease course. The skin findings in SLE are... (Review)
Review
The majority of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations at some point in their disease course. The skin findings in SLE are classified as SLE-specific or SLE-nonspecific based on histopathologic findings. SLE-specific skin diseases include chronic cutaneous lupus erythematosus (CLE), subacute CLE, and acute CLE. There are subsets of skin lesions within each group and the likelihood of associated SLE varies among them. SLE-nonspecific lesions are more common in patients with SLE and tend to coincide with active systemic disease. SLE-nonspecific lesions may be seen as a feature of another disease process, including other connective tissue diseases. It is important for the rheumatologist to be familiar with the spectrum of cutaneous diseases in SLE to help prognosticate the likelihood of systemic disease and to ensure patients receive timely dermatologic care with the goal of controlling disease activity to prevent damage.
Topics: Humans; Rheumatologists; Lupus Erythematosus, Cutaneous; Lupus Erythematosus, Systemic; Acute Disease; Motivation
PubMed: 36109075
DOI: 10.3899/jrheum.220089 -
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 -
Rheumatic Diseases Clinics of North... Feb 2018Gastrointestinal (GI) symptoms are common among patients with systemic lupus erythematosus (SLE), although only rarely are they caused by active organ system involvement... (Review)
Review
Gastrointestinal (GI) symptoms are common among patients with systemic lupus erythematosus (SLE), although only rarely are they caused by active organ system involvement from SLE itself. Rapid diagnosis and appropriate treatment of lupus enteritis and other GI manifestations of SLE are critical, because of the potential for organ and life-threatening complications. The 3 main variants of lupus enteritis are lupus mesenteric vasculitis, intestinal pseudo-obstruction, and protein-losing enteropathy. These GI manifestations and others in patients with SLE are reviewed here.
Topics: Disease Management; Early Diagnosis; Gastrointestinal Diseases; Gastrointestinal Tract; Humans; Lupus Erythematosus, Systemic
PubMed: 29149925
DOI: 10.1016/j.rdc.2017.09.011 -
Reumatologia Clinica 2015Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in... (Review)
Review
Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred.
Topics: Anti-Inflammatory Agents; Antiphospholipid Syndrome; Biological Factors; Cardiovascular Diseases; Disease Progression; Humans; Lupus Erythematosus, Systemic; Risk Factors; Vitamin D; Vitamins
PubMed: 25455719
DOI: 10.1016/j.reuma.2014.09.004 -
Frontiers in Immunology 2021Systemic lupus erythematosus (SLE), often considered the prototype of autoimmune diseases, is characterized by over-activation of the autoimmune system with abnormal... (Review)
Review
Systemic lupus erythematosus (SLE), often considered the prototype of autoimmune diseases, is characterized by over-activation of the autoimmune system with abnormal functions of innate and adaptive immune cells and the production of a large number of autoantibodies against nuclear components. Given the highly complex and heterogeneous nature of SLE, the pathogenesis of this disease remains incompletely understood and is presumed to involve both genetic and environmental factors. Currently, disturbance of the gut microbiota has emerged as a novel player involved in the pathogenesis of SLE. With in-depth research, the understanding of the intestinal bacteria-host interaction in SLE is much more comprehensive. Recent years have also seen an increase in metabolomics studies in SLE with the attempt to identify potential biomarkers for diagnosis or disease activity monitoring. An intricate relationship between gut microbiome changes and metabolic alterations could help explain the mechanisms by which gut bacteria play roles in the pathogenesis of SLE. Here, we review the role of microbiota dysbiosis in the aetiology of SLE and how intestinal microbiota interact with the host metabolism axis. A proposed treatment strategy for SLE based on gut microbiome (GM) regulation is also discussed in this review. Increasing our understanding of gut microbiota and their function in lupus will provide us with novel opportunities to develop effective and precise diagnostic strategies and to explore potential microbiota-based treatments for patients with lupus.
Topics: Animals; Bacteria; Disease Models, Animal; Dysbiosis; Gastrointestinal Microbiome; Humans; Lupus Erythematosus, Systemic
PubMed: 34335588
DOI: 10.3389/fimmu.2021.686501 -
Frontiers in Immunology 2021Animal models have played a crucial role in the understanding of the mechanisms and treatments of human diseases; however, owing to the large differences in genetic... (Review)
Review
Animal models have played a crucial role in the understanding of the mechanisms and treatments of human diseases; however, owing to the large differences in genetic background and disease-specific characteristics, animal models cannot fully simulate the occurrence and progression of human diseases. Recently, humanized immune system mice, based on immunodeficient mice, have been developed that allow for the partial reconstruction of the human immune system and mimic the human microenvironment. Systemic lupus erythematosus (SLE) is a complex disease characterized by the loss of tolerance to autoantigens, overproduction of autoantibodies, and inflammation in multiple organ systems. The detailed immunological events that trigger the onset of clinical manifestations in patients with SLE are still not well known. Two methods have been adopted for the development of humanized SLE mice. They include transferring peripheral blood mononuclear cells from patients with SLE to immunodeficient mice or transferring human hematopoietic stem cells to immunodeficient mice followed by intraperitoneal injection with pristane to induce lupus. However, there are still several challenges to be overcome, such as how to improve the efficiency of reconstruction of the human B cell immune response, how to extend the lifespan and improve the survival rate of mice to extend the observation period, and how to improve the development of standardized commercialized models and use them. In summary, there are opportunities and challenges for the development of humanized mouse models of SLE, which will provide novel strategies for understanding the mechanisms and treatments of SLE.
Topics: Animals; Autoantibodies; Autoantigens; Autoimmune Diseases; Autoimmunity; Biomarkers; Disease Management; Disease Models, Animal; Disease Susceptibility; Humans; Lupus Erythematosus, Systemic; Mice; Mice, Transgenic
PubMed: 35116040
DOI: 10.3389/fimmu.2021.816956 -
Trends in Molecular Medicine Feb 2021Systemic lupus erythematosus (SLE) is a multisystem, chronic autoimmune disease where treatment varies by patient and disease activity. Strong preclinical results and... (Review)
Review
Systemic lupus erythematosus (SLE) is a multisystem, chronic autoimmune disease where treatment varies by patient and disease activity. Strong preclinical results and clinical correlates have motivated development of many drugs, but many of these have failed to achieve efficacy in clinical trials. FDA approval of belimumab in 2011 was the first successful SLE drug in nearly six decades. In this article, we review insights into the molecular and clinical heterogeneity of SLE from transcriptomics studies and detail their potential impact on drug development and clinical practices. We critically examine the pipeline of SLE drugs, including past failures and their associated lessons and current promising approaches. Finally, we identify opportunities for integrating these findings and drug development with new multidisciplinary advances to enhance future SLE treatment.
Topics: Biological Variation, Population; Clinical Decision-Making; Disease Management; Disease Susceptibility; Drug Development; Humans; Lupus Erythematosus, Systemic; Molecular Targeted Therapy; Precision Medicine
PubMed: 33046407
DOI: 10.1016/j.molmed.2020.09.009 -
American Family Physician Dec 2010Careful examination of the oral cavity may reveal findings indicative of an underlying systemic condition, and allow for early diagnosis and treatment. Examination... (Review)
Review
Careful examination of the oral cavity may reveal findings indicative of an underlying systemic condition, and allow for early diagnosis and treatment. Examination should include evaluation for mucosal changes, periodontal inflammation and bleeding, and general condition of the teeth. Oral findings of anemia may include mucosal pallor, atrophic glossitis, and candidiasis. Oral ulceration may be found in patients with lupus erythematosus, pemphigus vulgaris, or Crohn disease. Additional oral manifestations of lupus erythematosus may include honeycomb plaques (silvery white, scarred plaques); raised keratotic plaques (verrucous lupus erythematosus); and nonspecific erythema, purpura, petechiae, and cheilitis. Additional oral findings in patients with Crohn disease may include diffuse mucosal swelling, cobblestone mucosa, and localized mucogingivitis. Diffuse melanin pigmentation may be an early manifestation of Addison disease. Severe periodontal inflammation or bleeding should prompt investigation of conditions such as diabetes mellitus, human immunodeficiency virus infection, thrombocytopenia, and leukemia. In patients with gastroesophageal reflux disease, bulimia, or anorexia, exposure of tooth enamel to acidic gastric contents may cause irreversible dental erosion. Severe erosion may require dental restorative treatment. In patients with pemphigus vulgaris, thrombocytopenia, or Crohn disease, oral changes may be the first sign of disease.
Topics: Bulimia; Early Diagnosis; Gastroesophageal Reflux; Humans; Lupus Erythematosus, Systemic; Periodontal Diseases; Thrombocytopenia; Tooth Erosion
PubMed: 21121523
DOI: No ID Found -
Clinical Reviews in Allergy & Immunology Dec 2018Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease with a prevalence of approximately 1 in 1000. Over the last 30 years, advances in treatment such... (Review)
Review
Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease with a prevalence of approximately 1 in 1000. Over the last 30 years, advances in treatment such as use of corticosteroids and immunosuppressants have improved life expectancy and quality of life for patients with lupus and the key unmet needs have therefore changed. With the reduced mortality from disease activity, development of cardiovascular disease (CVD) has become an increasingly important cause of death in patients with SLE. The increased CVD risk in these patients is partly, but not fully explained by standard risk factors, and abnormalities in the immune response to lipids may play a role. Invariant natural killer T cells, which are triggered specifically by lipid antigens, may protect against progression of subclinical atherosclerosis. However, currently our recommendation is that clinicians should focus on optimal management of standard CVD risk factors such as smoking, blood pressure and lipid levels. Fatigue is one of the most common and most limiting symptoms suffered by patients with SLE. The cause of fatigue is multifactorial and disease activity does not explain this symptom. Consequently, therapies directed towards reducing inflammation and disease activity do not reliably reduce fatigue and new approaches are needed. Currently, we recommend asking about sleep pattern, optimising pain relief and excluding other causes of fatigue such as anaemia and metabolic disturbances. For the subgroup of patients whose disease activity is not fully controlled by standard treatment regimes, a range of different biologic agents have been proposed and subjected to clinical trials. Many of these trials have given disappointing results, though belimumab, which targets B lymphocytes, did meet its primary endpoint. New biologics targeting B cells, T cells or cytokines (especially interferon) are still going through trials raising the hope that novel therapies for patients with refractory SLE may be available soon.
Topics: Biological Products; Cardiovascular Diseases; Clinical Trials as Topic; Comorbidity; Disease Management; Disease Susceptibility; Drug Resistance; Fatigue; Health Services Needs and Demand; Humans; Lupus Erythematosus, Systemic; Research; Risk Assessment; Risk Factors
PubMed: 28853005
DOI: 10.1007/s12016-017-8640-5