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REVIEW ARTICLE

Update on systemic lupus erythematosus: A review

Abdurahman Alloghbi1 ,  Yousef Al Obaisi2 ,  Hussain Alkhurayb2 ,  Amal M Shawabka3 ,  Fahad S. Alshehri2,4*
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1 Department of Oncology, College of Medicine, King Khalid University, Abha, Saudi Arabia
2 Pathology and Clinical Laboratory Medicine Administration, Haematology and Immunology Division, King Fahad Medical City, Riyadh, Saudi Arabia
3 Department of Clinical Science, College of Medicine, Palestine Polytechnic University, Hebron, Palestine
4 Department of Pathology and Clinical Laboratory Medicine, Haematology Division, King Faisal Medical City for Southern Region, Abha, Saudi Arabia
EJMO, 025090042 https://doi.org/10.36922/EJMO025090042
Received: 26 February 2025 | Revised: 5 May 2025 | Accepted: 8 May 2025 | Published online: 30 May 2025
© 2025 by the Author(s). This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution -Noncommercial 4.0 International License (CC-by the license) ( https://creativecommons.org/licenses/by-nc/4.0/ )
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Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune and idiopathic condition that affects connective tissues. It is characterized by the formation of autoantibodies, the accumulation of immune complexes, inflammation, and, ultimately, irreversible organ damage. It can affect individuals of various ages, ethnicities, and genders. Nonetheless, nearly 90% of newly diagnosed SLE patients are women of reproductive age. The early mortality associated with SLE is primarily due to significant disease activity. However, subsequent mortality is linked to chronic illness and the use of immunosuppressive agents. Despite extensive research, the precise etiology of SLE remains partially understood; however, its onset involves a complex interplay of genetic predispositions, environmental stimuli, hormonal factors, and immune system abnormalities. The predominant clinical signs include fatigue, fever, weight loss, arthritis, pericarditis, anemia, and pulmonary complications. Diagnosing SLE requires a combination of clinical evaluations and laboratory testing, including antinuclear antibodies and anti-double-stranded DNA antibodies, among others. Managing SLE patients involves various approaches and lifestyle recommendations, including avoiding direct or reflected sunlight. This article presents an updated review of the mortality and pathophysiology of SLE. We also elucidate the clinical manifestations and the primary diagnostic and therapeutic strategies for this condition.

Keywords
Systemic lupus erythematosus
Clinical manifestations
Mortality
Diagnosis
Antinuclear antibodies
Funding
None.
Conflict of interest
The authors declare that they have no competing interests.
References
  1. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-2121. doi: 10.1056/NEJMra1100359

 

  1. Isenberg D, Ehrenstein M, Rahman A. Introduction to a systemic lupus erythematosus - biomarkers meeting held at University College London on 23rd and 24th February 2007. Lupus. 2008;17(3):231. doi: 10.1177/0961203307088257

 

  1. Quirno N, Porrini A, Marino P, Iotti R. Causes of death in systemic lupus erythematosus. Prensa Med Argent. 1970. 57(22):1056-1059.

 

  1. Fei Y, Shi X, Gan F, et al. Death causes and pathogens analysis of systemic lupus erythematosus during the past 26 years. Clin Rheumatol. 2014;33(1):57-63. doi: 10.1007/s10067-013-2383-3

 

  1. Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Bucur). 2011;6(4):330-336.

 

  1. Bertsias GK, Salmon JE, Boumpas DT. Therapeutic opportunities in systemic lupus erythematosus: State of the art and prospects for the new decade. Ann Rheum Dis. 2010;69(9):1603-1611. doi: 10.1136/ard.2010.135186

 

  1. Stoddard CJ, Kay PH, Simms JM, Kennedy A, Hughes P. Acute abdominal complications of systemic lupus erythematosus. Br J Surg. 1978;65(9):625-628. doi: 10.1002/bjs.1800650911

 

  1. Palmer DG. Systemic lupus erythematosus with cerebral complications. N Z Med J. 1988;101(840):91.

 

  1. Pertschuk LP, et al. Acute pulmonary complications in systemic lupus erythematosus. Immunofluorescence and light microscopic study. Am J Clin Pathol. 1977;68(5):553-557. doi: 10.1093/ajcp/68.5.553

 

  1. Delaney P. Neurologic complications of systemic lupus erythematosus. Am Fam Physician. 1983;28(1):191-193.

 

  1. Mukherjee S. Long term complications in systemic lupus erythematosus. J Assoc Physicians India. 2006;54 Suppl:22-26.

 

  1. Moore JE, Shulman LE, Scott JT. The natural history of systemic lupus erythematosus: An approach to its study through chronic biologic false positive reactors: Interim report. Trans Am Clin Climatol Assoc. 1956;68:59-67; discussion 67-8.

 

  1. Mak A, Cheung MW, Chiew HJ, Liu Y, Ho RC. Global trend of survival and damage of systemic lupus erythematosus: Meta-analysis and meta-regression of observational studies from the 1950s to 2000s. Semin Arthritis Rheum. 2012;41(6):830-839. doi: 10.1016/j.semarthrit.2011.11.002

 

  1. Yen EY, Shaheen M, Woo JM, et al. 46-year trends in systemic lupus erythematosus mortality in the United States, 1968 to 2013: A nationwide population-based study. Ann Intern Med. 2017;167(11):777-785. doi: 10.7326/M17-0102

 

  1. Brinks R, Fischer-Betz R, Sander O, Richter JG, Chehab G, Schneider M. Age-specific prevalence of diagnosed systemic lupus erythematosus in Germany 2002 and projection to 2030. Lupus. 2014;23(13):1407-1411. doi: 10.1177/0961203314540352

 

  1. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: A comparison of worldwide disease burden. Lupus. 2006;15(5):308-318. doi: 10.1191/0961203306lu2305xx

 

  1. Wegman ME. Definition of mortality. Pediatrics. 1968;41(4):854-855.

 

  1. Kan HJ, Song X, Johnson BH, Bechtel B, O’Sullivan D, Molta CT. Healthcare utilization and costs of systemic lupus erythematosus in Medicaid. Biomed Res Int. 2013;2013:808391. doi: 10.1155/2013/808391

 

  1. Urowitz MB, Gladman DD. How to improve morbidity and mortality in systemic lupus erythematosus. Rheumatology (Oxford). 2000;39(3):238-244. doi: 10.1093/rheumatology/39.3.238

 

  1. Ling GS, Crawford G, Buang N, et al. C1q restrains autoimmunity and viral infection by regulating CD8(+) T cell metabolism. Science. 2018;360(6388):558-563. doi: 10.1126/science.aao4555

 

  1. Lee YJ, Park JA, Kwon H, et al. Role of stem cell-like memory T cells in systemic lupus erythematosus. Arthritis Rheumatol. 2018;70(9):1459-1469. doi: 10.1002/art.40524

 

  1. Fischer-Betz R, Herzer P, Schneider M. Systemic lupus erythematosus. Dtsch Med Wochenschr. 2005;130(43):2451- 8; quiz 2459-60, 2461-2.

 

  1. Ichikawa Y. Diagnosis and clinical symptoms of systemic lupus erythematosus and overlap syndrome. Nihon Naika Gakkai Zasshi. 1990;79(10):1360-1365.

 

  1. Horiuchi A. Diagnosis and therapy of systemic lupus erythematosus. Nihon Naika Gakkai Zasshi. 1993;82(3):381-5.

 

  1. Allard SA, Charles PJ, Herrick AL, McColl KE, Scott JT. Antinuclear antibodies and the diagnosis of systemic lupus erythematosus in patients with acute intermittent porphyria. Ann Rheum Dis. 1990;49(4):246-248. doi: 10.1136/ard.49.4.246

 

  1. Ho, A, Magder LS, Barr SG, Petri M. Decreases in anti-double-stranded DNA levels are associated with concurrent flares in patients with systemic lupus erythematosus. Arthritis Rheum. 2001;44(10):2342-2349. doi: 10.1002/1529-0131(200110)44:10<2342:aid-art397>3.0.co;2-8

 

  1. Smeenk R, Brinkman K, Van den Brink H, et al. Antibodies to DNA in patients with systemic lupus erythematosus. Their role in the diagnosis, the follow-up and the pathogenesis of the disease. Clin Rheumatol. 1990;9(1 Suppl):100-110. doi: 10.1007/BF02205557

 

  1. Ho A, Barr SG, Magder LS, Petri M. A decrease in complement is associated with increased renal and hematologic activity in patients with systemic lupus erythematosus. Arthritis Rheum. 2001;44(10):2350-2357. doi: 10.1002/1529-0131(200110)44:10<2350:aid-art398>3.0.co;2-a

 

  1. Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A. Diagnosis, monitoring, and treatment of systemic lupus erythematosus: A systematic review of clinical practice guidelines. Arthritis Care Res (Hoboken). 2015;67(10):1440-1452. doi: 10.1002/acr.22591

 

  1. Alabduljabbar K, Alkhalifah M, Aldheshe A, et al. Development of a cardiovascular disease risk prediction model: A preliminary retrospective cohort study of a patient sample in Saudi Arabia. J Clin Med. 2023;12(15):5115. doi: 10.3390/jcm12155115

 

  1. Tash AA, Al-Bawardy RF. Cardiovascular disease in Saudi Arabia: Facts and the way forward. J Saudi Heart Assoc. 2023;35(2):148-162. doi: 10.37616/2212-5043.1336

 

  1. Alenzi EO, Fatima W, Amara A, et al. A systematic review of chronic diseases and their prevalence among the population of northern borders province (NBP) in Saudi Arabia. J Multidiscip Healthc. 2023;16:1047-1056. doi: 10.2147/JMDH.S401001

 

  1. Alibrahim B, Sharaheeli J, Alassaf L, Abd-Ellatif EE. Risk assessment of cardiovascular disease among adults attending primary healthcare centers in Riyadh City 2015. Cureus. 2022;14(12):e32503. doi: 10.7759/cureus.32503

 

  1. Ghamri RA, Alzahrani NS, Alharthi AM, Gadah HJ, Badoghaish BG, Alzahrani AA. Cardiovascular risk factors among high-risk individuals attending the general practice at king Abdulaziz University hospital: A cross-sectional study. BMC Cardiovasc Disord. 2019;19(1):268. doi: 10.1186/s12872-019-1261-6

 

  1. Criswell LA. The genetic contribution to systemic lupus erythematosus. Bull NYU Hosp Jt Dis. 2008;66(3):176-183.

 

  1. International Consortium for Systemic Lupus Erythematosus Genetics (SLEGEN), Harley JB, Alarcón-Riquelme ME, et al. Genome-wide association scan in women with systemic lupus erythematosus identifies susceptibility variants in ITGAM, PXK, KIAA1542 and other loci. Nat Genet. 2008;40(2):204-210. doi: 10.1038/ng.81

 

  1. Barbhaiya M, Costenbader KH. Ultraviolet radiation and systemic lupus erythematosus. Lupus. 2014;23(6):588-595. doi: 10.1177/0961203314530488

 

  1. James JA, Kaufman KM, Farris AD, Taylor-Albert E, Lehman TJ, Harley JB. An increased prevalence of Epstein- Barr virus infection in young patients suggests a possible etiology for systemic lupus erythematosus. J Clin Invest. 1997;100(12):3019-3026. doi: 10.1172/JCI119856

 

  1. Karpuzoglu E, Zouali M. The multi-faceted influences of estrogen on lymphocytes: Toward novel immuno-interventions strategies for autoimmunity management. Clin Rev Allergy Immunol. 2011;40(1):16-26. doi: 10.1007/s12016-009-8188-0

 

  1. Gazy NE, Fewings NL, Emerson J, Swaminathan S, Lee AY, Lin MW. Interferon signatures in systemic lupus erythematosus patients across ethnic groups. Lupus. 2023;32(10):1231-1232. doi: 10.1177/09612033231194211

 

  1. Vollmer J, Tluk S, Schmitz C, et al. Immune stimulation mediated by autoantigen binding sites within small nuclear RNAs involves Toll-like receptors 7 and 8. J Exp Med. 2005;202(11):1575-1585. doi: 10.1084/jem.20051696

 

  1. Tanaka Y, Kusuda M, Yamaguchi Y. Interferons and systemic lupus erythematosus: Pathogenesis, clinical features, and treatments in interferon-driven disease. Mod Rheumatol. 2023;33(5):857-867. doi: 10.1093/mr/roac140

 

  1. Baechler EC, Batliwalla FM, Karypis G, et al. Interferon-inducible gene expression signature in peripheral blood cells of patients with severe lupus. Proc Natl Acad Sci U S A. 2003;100(5):2610-2615. doi: 10.1073/pnas.0337679100

 

  1. Tran DQ. In vitro suppression assay for functional assessment of human regulatory T cells. Methods Mol Biol. 2013;979:199-212. doi: 10.1007/978-1-62703-290-2_16

 

  1. Postal M, Peliçari KO, Sinicato NA, Marini R, Costallat LT, Appenzeller S. Th1/Th2 cytokine profile in childhood-onset systemic lupus erythematosus. Cytokine. 2013;61(3):785-791. doi: 10.1016/j.cyto.2012.11.023

 

  1. Longhi MS, Ma Y, Grant CR, et al. T-regs in autoimmune hepatitis-systemic lupus erythematosus/mixed connective tissue disease overlap syndrome are functionally defective and display a Th1 cytokine profile. J Autoimmun. 2013;41:146-151. doi: 10.1016/j.jaut.2012.12.003

 

  1. McKinley PS, Ouellette SC, Winkel GH. The contributions of disease activity, sleep patterns, and depression to fatigue in systemic lupus erythematosus. A proposed model. Arthritis Rheum. 1995;38(6):826-834. doi: 10.1002/art.1780380617

 

  1. Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: A comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore). 2003;82(5):299-308. doi: 10.1097/01.md.0000091181.93122.55

 

  1. Greco CM, Rudy TE, Manzi S. Adaptation to chronic pain in systemic lupus erythematosus: Applicability of the multidimensional pain inventory. Pain Med. 2003;4(1):39-50. doi: 10.1046/j.1526-4637.2003.03001.x

 

  1. Miner JJ, Kim AH. Cardiac manifestations of systemic lupus erythematosus. Rheum Dis Clin North Am. 2014;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003

 

  1. Richter JG, Sander O, Schneider M, Klein-Weigel P. Diagnostic algorithm for Raynaud’s phenomenon and vascular skin lesions in systemic lupus erythematosus. Lupus. 2010;19(9):1087-1095. doi: 10.1177/0961203310374304

 

  1. Barile-Fabris L, Hernandez-Cabrera MF, Barragan- Garfias JA. Vasculitis in systemic lupus erythematosus. Curr Rheumatol Rep. 2014;16(9):440. doi: 10.1007/s11926-014-0440-9

 

  1. Sarabi ZS, Chang E, Bobba R, et al. Incidence rates of arterial and venous thrombosis after diagnosis of systemic lupus erythematosus. Arthritis Rheum. 2005;53(4):609-612. doi: 10.1002/art.21314

 

  1. Danila MI, Pons-Estel GJ, Zhang J, Vilá LM, Reveille JD, Alarcón GS. Renal damage is the most important predictor of mortality within the damage index: Data from LUMINA LXIV, a multiethnic US cohort. Rheumatology (Oxford). 2009;48(5):542-545. doi: 10.1093/rheumatology/kep012

 

  1. Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: Insight into pathogenesis, diagnosis and treatment. World J Gastroenterol. 2010;16(24):2971-2977. doi: 10.3748/wjg.v16.i24.2971

 

  1. Romero-Diaz J, Garcia-Sosa I, Sanchez-Guerrero J. Thrombosis in systemic lupus erythematosus and other autoimmune diseases of recent onset. J Rheumatol. 2009;36(1):68-75. doi: 10.3899/jrheum.071244

 

  1. Newman K, Owlia MB, El-Hemaidi I, Akhtari M. Management of immune cytopenias in patients with systemic lupus erythematosus - old and new. Autoimmun Rev. 2013;12(7):784-791. doi: 10.1016/j.autrev.2013.02.001

 

  1. Silpa-Archa S, Lee JJ, Foster CS. Ocular manifestations in systemic lupus erythematosus. Br J Ophthalmol. 2016;100(1):135-141. doi: 10.1136/bjophthalmol-2015-306629

 

  1. Gallais Serezal I, Bouillet L, Dhôte R, et al. Hereditary angioedema and lupus: A French retrospective study and literature review. Autoimmun Rev. 2015;14(6):564-568. doi: 10.1016/j.autrev.2015.02.001

 

  1. Abu-Shakra M, Gladman DD, Urowitz MB, Farewell V. Anticardiolipin antibodies in systemic lupus erythematosus: Clinical and laboratory correlations. Am J Med. 1995;99(6):624-628. doi: 10.1016/s0002-9343(99)80249-6

 

  1. Haliloglu S, Carlioglu A, Akdeniz D, Karaaslan Y, Kosar A. Fibromyalgia in patients with other rheumatic diseases: Prevalence and relationship with disease activity. Rheumatol Int. 2014;34(9):1275-1280. doi: 10.1007/s00296-014-2972-8

 

  1. Ferrari SM, Elia G, Virili C, Centanni M, Antonelli A, Fallahi P. Systemic lupus erythematosus and thyroid autoimmunity. Front Endocrinol (Lausanne). 2017;8:138. doi: 10.3389/fendo.2017.00138

 

  1. Armas Cruz R. Diagnosis of systemic lupus erythematosus (SLE). Rev Med Chil. 1967;95(10):628-641.

 

  1. Gilboe IM, Husby G. Application of the 1982 revised criteria for the classification of systemic lupus erythematosus on a cohort of 346 Norwegian patients with connective tissue disease. Scand J Rheumatol. 1999;28(2):81-87. doi: 10.1080/030097499442531

 

  1. Pincus T. A pragmatic approach to cost-effective use of laboratory tests and imaging procedures in patients with musculoskeletal symptoms. Prim Care. 1993;20(4):795-814.

 

  1. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271-1277. doi: 10.1002/art.1780251101

 

  1. Brydak-Godowska J. General and ocular symptoms in systemic lupus erythematosus (SLE). Klin Oczna. 2007;109(1-3):68-70.

 

  1. Attman PO, Larsson O, Westberg G. Diagnosis, therapy and follow-up of patients with SLE. Lakartidningen. 1978;75:2218-2219.

 

  1. Villalta D, Romelli PB, Savina C, et al. Anti-dsDNA antibody avidity determination by a simple reliable ELISA method for SLE diagnosis and monitoring. Lupus. 2003;12(1):31-36. doi: 10.1191/0961203303lu277oa

 

  1. Londhey VA. Anti-nucleosome antibodies: Utility in diagnosis of SLE and monitoring disease activity. J Assoc Physicians India. 2013;61(6):369-370.

 

  1. Alba P, Bertolaccini ML, Khamashta MA. The use of laboratory methods in differential diagnosis and treatment of SLE and antiphospholipid syndrome. Expert Rev Clin Immunol. 2007;3(4):613-622. doi: 10.1586/1744666X.3.4.613

 

  1. Tamburino C, Fiore CE, Foti R, Salomone E, Di Paola R, Grimaldi DR. Endomyocardial biopsy in diagnosis and management of cardiovascular manifestations of systemic lupus erythematosus (SLE). Clin Rheumatol. 1989;8(1):108-112. doi: 10.1007/BF02031079

 

  1. Yasue T, Akutsu J, Shinjo H, Sasada K. The significance of serum concentration of C3 and C4 for the diagnosis, prognosis and treatment of SLE. Nihon Hifuka Gakkai Zasshi. 1975;85(14):875-83.

 

  1. Hess C. Systemic lupus erythematosus (SLE)--a brief guide to its diagnosis. Ther Umsch. 2005;62(5):297-301. doi: 10.1024/0040-5930.62.5.297

 

  1. Bravo MG, Alarcon-Segovia D. C-reactive protein in the differential diagnosis between infection and disease reactivation in SLE. J Rheumatol. 1981;8(2):291-294.

 

  1. Egner W. The use of laboratory tests in the diagnosis of SLE. J Clin Pathol. 2000;53(6):424-432. doi: 10.1136/jcp.53.6.424

 

  1. Lehmann P, Homey B. Clinic and pathophysiology of photosensitivity in lupus erythematosus. Autoimmun Rev. 2009;8(6):456-461. doi: 10.1016/j.autrev.2008.12.012

 

  1. Cai T, Zhao J, Yang Y, Jiang Y, Zhang JA. Hydroxychloroquine use reduces mortality risk in systemic lupus erythematosus: A systematic review and meta-analysis of cohort studies. Lupus. 2022;31(14):1714-1725. doi: 10.1177/09612033221129774

 

  1. Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: A systematic review. Ann Rheum Dis. 2010;69(1):20-28. doi: 10.1136/ard.2008.101766

 

  1. Belmon HM. Treatment of systemic lupus erythematosus - 2013 update. Bull Hosp Jt Dis (2013). 2013;71(3):208-213.

 

  1. Tanaka Y. State-of-the-art treatment of systemic lupus erythematosus. Int J Rheum Dis. 2020;23(4):465-471. doi: 10.1111/1756-185X.13817

 

  1. Merrill JT, Furie R, Werth VP, et al. Anifrolumab effects on rash and arthritis: Impact of the type I interferon gene signature in the phase IIb MUSE study in patients with systemic lupus erythematosus. Lupus Sci Med. 2018;5(1):e000284. doi: 10.1136/lupus-2018-000284

 

  1. Singh JA, Shah NP, Mudano AS. Belimumab for systemic lupus erythematosus. Cochrane Database Syst Rev. 2021;2(2):CD010668. doi: 10.1002/14651858.CD010668.pub2

 

  1. Leandro M, Isenberg DA. Rituximab - the first twenty years. Lupus. 2021;30(3):371-377. doi: 10.1177/096120332098266
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