While the incidence of SLE and lupus nephritis remained stable over the study period (19992013), prevalence increased. One hypothesis focuses on the roles of estrogens, progesterone, testosterone, dehydroepiandrosterone (DHEA), and prolactin in immune system responsiveness. Hormonal regulation of B-cell function and systemic lupus erythematosus. Incidence of systemic lupus erythematosus in a population-based cohort using revised 1997 American College of Rheumatology and the 2012 Systemic Lupus International Collaborating Clinics classification criteria. Rheumatology 53, 180185 (2014). PubMed SLE doubles the risk of stroke with the highest relative risk for of stroke observed within the first year after diagnosis. Online ahead of print. & Jawad, A. S. The effect of ethnicity and genetic ancestry on the epidemiology, clinical features and outcome of systemic lupus erythematosus. 46-year trends in systemic lupus erythematosus mortality in the United States, 1968 to 2013: a nationwide population-based study. Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada, Department of Medicine, Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, USA, Department of Anesthesiology, Perioperative and Pain Medicine, Stanford School of Medicine, Stanford, CA, USA, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Victoria, Australia, Division of Rheumatology, University Medicine Cluster, National University Health System, Singapore, Singapore, Department of Medicine, Yong Loo Lin School of Medicine, National University Hospital, Singapore, Singapore, Department of Medicine Solna, Unit of Rheumatology, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden, Galter Health Sciences Library and Learning Center, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA, Department of Medicine, Rheumatology Division, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA, You can also search for this author in Bookshelf Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that causes inflammation of connective tissues [].In general, SLE has propensity to affect every organ and tissue of the body, and its pattern of clinical manifestations varies widely among patients [].The complex interactions among genetic disposition, environmental risk factors, and the hormonal status . Internationally, the frequent causes of death for patients with SLE include infection and cardiovascular disease. The site is secure. The estimated incidence, prevalence and mortality of systemic lupus erythematosus (SLE) vary considerably between geographic regions. and transmitted securely. The approach to the treatment of signs and symptoms of lupus depends on the type and the severity of disease. Cardiac wall rupture in systemic lupus erythematosus: a case report and review of the literature. Arthritis Rheumatol. Unable to load your collection due to an error, Unable to load your delegates due to an error. Most cases of lupus are believed to be caused by changes in multiple genes (called polygenic), but some are associated with single mutations, which is called monogenic. made substantial contributions to discussions of the content; M.R.W.B., C.D., T.F., A.H., A.M., N.Y.K. Hart, H. H., Grigor, R. R. & Caughey, D. E. Ethnic difference in the prevalence of systemic lupus erythematosus. Deficient CD4 + CD25 (high) T regulatory cell function in patients with active systemic lupus erythematosus. Furst et al. Clin. All registries defined cases of SLE according to the American College of Rheumatology (ACR) 1997 revised classification criteria for SLE. Ann. In: Firestein GS, Budd RC, Harris ED Jr, et al., editors. Tektonidou, M. G., Lewandowski, L. B., Hu, J., Dasgupta, A. & Jonsen, A. An official website of the United States government. Testing relationship between tea intake and the risk of rheumatoid arthritis and systemic lupus erythematosus: a Mendelian randomization study, Immunomodulatory effects of atorvastatin on MRL/lpr mice, Risk of Severe COVID-19 Outcomes Among Patients with Immune-Mediated Inflammatory Diseases or Malignancies: A Retrospective Analysis of Real-World Data in the United States, https://doi.org/10.1038/s41584-021-00690-3. Caucasian (n = 294), Asian/Pacific Islander (n = 290), African American (n = 160), Hispanic (n=118), American Indian/Alaskan native (n = 4); 307 non-Hispanic white, 282 non-Hispanic black, 344 Hispanic, 111 non-Hispanic Asian, Hospital records, laboratory results, histopathology, Diagnosis registries, hospital records, laboratory databases. Dis. Collaborators, G. B. D. R. F. Global burden of 87 risk factors in 204 countries and territories, 19902019: a systematic analysis for the Global Burden of Disease Study 2019. K01 AR079039/AR/NIAMS NIH HHS/United States. The T-cell receptor binds to the major histocompatibility complex (MHC) portion of the APC, which may lead to cytokine release, inflammation, and B-cell stimulation.12 Stimulation of B-cell division and the production of immunoglobulin G (IgG) autoantibodies that can cause tissue damage also occur in SLE.12,1519 Unlike the situation in healthy adults, autoantigen-specific T cells and B cells may also interact and produce harmful autoantibodies.12,20, Many of the autoantibodies identified in SLEthe antinuclear antibodies (ANAs)target nuclear components of cells. Associations between lupus onset and age, sex, geography, and race have also been established. Lupus 29, 1526 (2020). Onset age affects mortality and renal outcome of female systemic lupus erythematosus patients: a nationwide population-based study in Taiwan. Arthritis Rheumatol. Several studies examined the malignancy risk among SLE patients. Joo, Y. 4 UNI | 4.95 per 1UNI. PubMed Central Lupus 25, 772780 (2016). 18, 117128 (2015). Valencia X, Yarboro C, Illei G, Lipsky PE. DLE is manifested as a chronic scarring and atrophic photosensitive dermatosis, which may progress to SLE or may occur in patients with SLE. Large-scale mortality gap between SLE and control population is associated with increased infection-related mortality in lupus. Guidelines for referral and management of systemic lupus erythematosus in adults. One study revealed concordance rates of 14% to 57% in identical twins sharing the same trait; a second study showed an incidence rate of 24% to 58%.2729 In another study of non-identical twins, concordance rates of 3% to 10% were documented.29, The investigation of a genetic influence on SLE has led to the discovery of a number of gene variants linked to SLE expression. Nowling TK, Gilkeson GS. Most steroids are Pregnancy Category C drugs. Lupus 28, 16101611 (2019). Musculoskeletal involvement includes arthralgias, myalgias, and/or arthritis. CAS Lawrence RC, Helmick CG, Arnett FC, et al. Chemical and physical sunblocks, topical corticosteroids, or antimalarial agents are commonly used to prevent disease flares and to manage the clinical manifestations associated with DLE. Lin, H. et al. J. An official website of the United States government. Age-specific and sex-specific incidence of systemic lupus erythematosus: an estimate from cross-sectional claims data of 2.3 million people in the German statutory health insurance 2002. Global incidence estimates for SLE. Clin. Korean J. Intern. FOIA Fatoye, F., Gebrye, T. & Svenson, L. W. Real-world incidence and prevalence of systemic lupus erythematosus in Alberta, Canada. 212. N. Z. J. Med. Rees F, Doherty M, Grainge M, Davenport G, Lanyon P, Zhang W. The incidence and prevalence of systemic lupus erythematosus in the UK, 19992012. Arthritis Care Res. Drug-induced lupus. The incidence and prevalence of systemic lupus erythematosus, 20022004: the Georgia Lupus Registry. Discoid lupus erythematosus as part of a larger disease spectrum: Correlation of clinical features with laboratory findings in lupus erythematosus. Wu, X. Y. et al. Foocharoen, C., Nanagara, R., Suwannaroj, S. & Mahakkanukrauh, A. Systemic lupus erythematosus (SLE) is the prototypical systemic autoimmune disease with a significant disease burden across the world among different ethnic, racial and age groups. During the process of cellular death, pieces of cellular material form on the surface of the dying cell. Patients with SLE have higher titers of antibodies to EBV.40 Smoking, silica, and some hair products (e.g., dyes) may also be possible triggers of lupus. Unlike that of idiopathic SLE, the incidence of DIL is similar among men and women; the disease primarily affects patients of advanced age.3,63, The exact cause of DIL is unknown, but genetics are believed to be involved. An increased prevalence of EpsteinBarr virus infection in young patients suggests a possible etiology for systemic lupus erythematosus. Leuchten, N. et al. Lupus 20, 13291335 (2011). Common clinical presentations involve the heart, liver, and skin. The figure shows the reported prevalence ranges for systemic lupus erythematosus (SLE) per location (per 100,000 of the population), as denoted by the key. 69, 19962005 (2017). All other authors declare no competing interests. Not all data have been collected and reported uniformly across global regions. N. Z. J. Med. 69, 20062017 (2017). Association between prolactin and disease activity in systemic lupus erythematosus: Influence of statistical power. A second example of genetic variance is a possible deficiency of the C4 complement, a component identified in the elimination of self-reactive B cells. Rheumatology (Oxford). Performance of antinuclear antibodies for classifying systemic lupus erythematosus: a systematic literature review and meta-regression of diagnostic data. Was 21.99. Article A common cause of late mortality related to SLE is an accelerated atherosclerosis that is associated with either the disease or the treatment.9, SLE is a chronic disease that affects various organ systems, primarily as a consequence of the formation and deposition of autoantibodies and immune complexes, leading to eventual organ damage. Ingvarsson RF, Bengtsson AA, Jnsen A. Dis. Mok CC, Lau CS. LinkedIn is the world's largest business network, helping professionals like Thomas Annet discover inside connections to recommended job candidates, industry experts, and business partners. Klinefelters syndrome, which features an extra X chromosome in males, is linked to an elevated incidence of SLE, thereby providing further support for the association between SLE and a possible hormonal pathogenesis.50, The presentation of SLE can be complex, considering the number of organ systems that can be affected by the disease. FOIA Monogenic Lupus. Other examples of ANAs are the anti-Ro and anti-La antibodies that, when detected during pregnancy, have been linked to fetal heart damage as well as the anti-Smith (Sm) antibodies, which are a marker of kidney disease.2224, A second grouping of autoantibodies targets the phospholipid moiety of the prothrombin activator complex as well as cardiolipin. Rheumatol. Rheumatol. Baseline and monthly monitoring (e.g., laboratory tests, ultrasonography, fetal surveillance tests, maternal echocardiography, and antibody testing) should be performed for all pregnant lupus patients, because signs and symptoms of lupus flares may be similar to those typical of pregnancy.92 Neonates should be carefully evaluated for placental transfer of maternal antibodies, which could lead to cutaneous or cardiac complications (e.g., congenital heart block and cardiomyopathy).93. doi: 10.2196/44660. Intern. Maternal autoantibodies and congenital heart block: Mediators, markers, and therapeutic approach. Bae, S. C., Fraser, P. & Liang, M. H. The epidemiology of systemic lupus erythematosus in populations of African ancestry: a critical review of the prevalence gradient hypothesis. Hwang, J. et al. The .gov means its official. Deapen D, Escalante A, Weinrib L, et al. The incidence and prevalence of systemic lupus erythematosus in the UK, 19992012. Open J. Rheumatol. Federal government websites often end in .gov or .mil. Google Scholar. Hyperactive B cells, resulting from T-cell and antigen stimulation, increase the production of these antibodies against antigens that are exposed on the surface of apoptotic cells.12, The antigens causing T-cell and B-cell stimulation in patients with SLE can be attributed to the inappropriate disposal of apoptotic cells. Such data are currently lacking because of potential obstacles, such as differing case definitions, small-source populations, and varying demographic group targets.47, SLE is more common in women, particularly those of child-bearing age. Izmirly PM, Llanos C, Lee LA, et al. Yen, E. Y. et al. Health Sci. The socioeconomic burden of SLE. In the microvasculature, these complexes induce inflammatory reactions, causing the tissue inflammation and damage associated with SLE. Among the 86 patients who met inclusion criteria, 12 had systemic lupus erythematosus. 38, 107115 (2019). 66, 630637 (2015). The prevalence estimate was 9 times higher among females than among males (128.7 versus 14.6 per 100,000), and highest among Black females (230.9 per 100,000), followed by Hispanic females (120.7 per 100,000), White females (84.7 per 100,000), and Asian/Pacific Islander females (84.4 per 100,000). 34, 909917 (2014). Hochberg MC. Ann. Nonsteroidal anti-inflammatory drugs (NSAIDs) help to relieve musculoskeletal symptoms. Arthritis Care Res. National Library of Medicine Coffman RL, Lebman DA, Rothman P. Mechanism and regulation of immunoglobulin isotype switching. Gban-Kon, M. et al. Mild-to-moderate infusion reactions were reported most often.85,86, A few randomized controlled studies have provided mixed results regarding the efficacy and role of rituximab in the treatment of SLE. Borchers AT, Keen CL, Gershwin ME. 2023 Mar 29;25:e44660. Immunosuppressants were used more frequently by children with SLE. Costenbader KH, Feskanich D, Stampfer MJ, Karlson EW. Global epidemiology of systemic lupus erythematosus. Hochberg MC. Fig. Long-term outcome in Japanese patients with lupus nephritis. This study included 20,045 participants aged 17 years and older from the Third National Health and Nutritional Examination Survey (NHANES III) from 1988 to 1994. The severity of SLE did not change during the study period. Am. 28, 519524 (2010). Mechanism of immunosuppression of progesterone on maternal lymphocyte activation during pregnancy. Hermansen, M. L., Lindhardsen, J., Torp-Pedersen, C., Faurschou, M. & Jacobsen, S. The risk of cardiovascular morbidity and cardiovascular mortality in systemic lupus erythematosus and lupus nephritis: a Danish nationwide population-based cohort study. Arthritis Rheumatol. Klinefelters syndrome (47, XXY) in male systemic lupus erythematosus patients: Support for the notion of a genedose effect from the X chromosome. Cardiovascular effects often include pericarditis, myocarditis, endocarditis, and coronary artery disease.52,53 It has been theorized that certain drugs used to treat SLE (e.g., immunosuppressants and corticosteroids) are risk factors for coronary artery disease in SLE patients along with the traditional risk factors observed in the general population.52, Signs of gastrointestinal involvement include nausea, vomiting, and abdominal pain. Epidemiology and burden of systemic lupus erythematosus in a Southern European population: data from the community-based lupus registry of Crete, Greece. Demographics and laboratory test . Procainamide (e.g., Pronestyl, Bristol-Myers Squibb) and hydralazine (e.g., Apresoline, Novartis), although not frequently used today, have been commonly associated with DIL.67,68 Penicillamine (e.g., Cuprimine, Merck), minocycline (Minocin Wyeth/Pfizer), isoniazid (formerly Nydrazid, no longer available in the U.S.), methyldopa (e.g., Aldomet, Merck), and antitumor necrosis factor (anti-TNF) agents have also been linked to DIL. This Review focuses primarily on the past 5 years of global epidemiological studies and discusses the regional incidence and prevalence of SLE and top causes of mortality. C.D. sharing sensitive information, make sure youre on a federal Open Access Radiography can be used to assess joint involvement; renal ultrasound, kidney size and impairment; chest radiography, pulmonary involvement; and electrocardiography, chest pain. 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