Systemic Lupus Erythematosus Disease: An Overview of the Clinical Approach to Pathogenesis, Diagnosis, and Treatment
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Submitted
28 December 2020
Published
30 May 2021
Keywords:
-
Systemic Lupus Erythematosus, Autoimmune disease, Inflammatory disorder
Abstract
The systemic lupus erythematosus (SLE), commonly known as Lupus, is a rare and complex multisystem autoimmune disease where one’s immune system is overactive, and the body attacks its organ systems. SLE is a historically old disease described already in antiquity; it is an example of a chronic disease with physical, psychological, financial, and social implications for individuals diagnosed. It has inspired medical and basic biological scientists that focus on molecular biology, basic immunology, immunopathology, clinical science, genetics, and epidemiology. The syndrome is real in its existence-although hidden behind obstacles, cumbersome for patients and clinicians, and rebellious for scientists. There is currently no cure for SLE. The goal of treatment is to ease symptoms. This article will review information on the general approach to SLE therapy, focusing on currently approved therapies and novel approaches that might be used in the future.
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References
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12. Fatoye F, Gebrye T, Svenson LW. Real-world incidence and prevalence of systemic lupus erythematosus in Alberta, Canada. Rheumatol Int. 2018;38(9):1721-6. doi:10.1007/s00296-018-4091-4
13. Bharath G, Kumar P, Makkar N, Singla P, Soneja M, Biswas A, et al. Mortality in systemic lupus erythematosus at a teaching hospital in India: A 5-year retrospective study. J Family Med Prim Care. 2019;8(7):2511-5. doi:10.4103/jfmpc.jfmpc_362_19
14. Osio-Salido E, Manapat-Reyes H. Epidemiology of systemic lupus erythematosus in Asia. Lupus. 2010;19(12):1365-73. doi:10.1177/0961203310374305
15. Gergianaki I, Bertsias G. Systemic Lupus Erythematosus in Primary Care: An Update and Practical Messages for the General Practitioner. Front Med. 2018;5:161. doi:10.3389/fmed.2018.00161
16. Williams EM, Bruner L, Adkins A, Vrana C, Logan A, Kamen D, et al. I too, am America: a review of research on systemic lupus erythematosus in African-Americans. Lupus Sci Med. 2016;3(1):e000144. doi:10.1136/lupus-2015-000144
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18. Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices. Kidney Int. 2018;93(4):789-96. doi:10.1016/j.kint.2017.11.023
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20. Hon KL, Leung AKC. Neonatal Lupus Erythematosus. Autoimmune Dis. 2012;2012:301274. doi:10.1155/2012/301274
21. Costagliola G, Mosca M, Migliorini P, Consolini R. Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood. Front Pediatr. 2018;6:144. doi:10.3389/fped.2018.00144
22. Okon LG, Werth VP. Cutaneous Lupus Erythematosus: Diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27(3):391-404. doi:10.1016/j.berh.2013.07.008
23. Sarzi-Puttini P, Atzeni F, Capsoni F, Lubrano E, Doria A. Drug-induced lupus erythematosus. Autoimmunity. 2005;38(7):507-18. doi:10.1080/08916930500285857
24. Rekvig OP. Systemic Lupus Erythematosus: Definitions, Contexts, Conflicts, Enigmas. Front Immunol. 2018;9:387. doi:10.3389/fimmu.2018.00387
25. Moulton VR, Suarez-Fueyo A, Meidan E, Li H, Mizui M, Tsokos GC. Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective. Trend Mol Med. 2017;23(7):615-35. doi:10.1016/j.molmed.2017.05.006
26. Barbhaiya M, Costenbader KH. Environmental Exposures and the Development of Systemic Lupus Erythematosus. Curr Opin Rheumatol. 2016;28(5):497-505. doi:10.1097/BOR.0000000000000318
27. Arneth B. Systemic Lupus Erythematosus and DNA Degradation and Elimination Defects. Front Immunol. 2019;10:1697. doi:10.3389/fimmu.2019.01697
28. Zharkova O, Celhar T, Cravens PD, Satterthwaite AB, Fairhurst AM, Davis LS. Pathways leading to an immunological disease: systemic lupus erythematosus. Rheumatology. 2017;56(suppl_1):i55-i66. doi:10.1093/rheumatology/kew427
29. Pan Q, Chen J, Guo L, Lu X, Liao S, Zhao C, et al. Mechanistic insights into environmental and genetic risk factors for systemic lupus erythematosus. Am J Transl Res. 2019;11(3):1241-54.
30. Pan L, Lu MP, Wang JH, Xu M, Yang SR. Immunological pathogenesis and treatment of systemic lupus erythematosus. World J Pediatr. 2020;16(1):19-30. doi:10.1007/s12519-019-00229-3
31. Liao X, Reihl AM, Luo XM. Breakdown of Immune Tolerance in Systemic Lupus Erythematosus by Dendritic Cells. J Immunol Res. 2016;2016:6269157. doi:10.1155/2016/6269157
32. Avasare RS, Yee J. Lupus Nephritis: Breaking the Lull. Adv Chronic Kidney Dis. 2019;26(5):307-10. doi:10.1053/j.ackd.2019.05.005
33. Kuhn A, Bonsmann G, Anders HJ, Herzer P, Tenbrock K, Schneider M. The Diagnosis and Treatment of Systemic Lupus Erythematosus. Dtsch Arztebl Int. 2015;112(25):423-32. doi:10.3238/arztebl.2015.0423
34. Aringer M, Leuchten N, Johnson SR. New Criteria for Lupus. Curr Rheumatol Rep. 2020;22(6):18. doi:10.1007/s11926-020-00896-6
35. Vasquez-Canizares N, Wahezi D, Putterman C. Diagnostic and Prognostic Tests in Systemic Lupus Erythematosus. Best Pract Res Clin Rheumatol. 2017;31(3):351-63. doi:10.1016/j.berh.2017.10.002
36. Adamichou C, Bertsias G. Flares in systemic lupus erythematosus: diagnosis, risk factors and preventive strategies. Mediterr J Rheumatol. 2017;28(1):4-12. doi:10.31138/mjr.28.1.4
37. Lam NCV, Ghetu MV, Bieniek ML. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician. 2016;94(4):284-94.
38. Basta F, Fasola F, Triantafyllias K, Schwarting A. Systemic Lupus Erythematosus (SLE) Therapy: The Old and the New. Rheumatol Ther. 2020;7(3):433-46. doi:10.1007/s40744-020-00212-9
39. Ruiz-Irastorza G, Bertsias G. Treating systemic lupus erythematosus in the 21st century: new drugs and new perspectives on old drugs. Rheumatology. 2020;59(Suppl 5):v69-v81. doi:10.1093/rheumatology/keaa403
40. Durcan L, O’Dwyer T, Petri M. Management strategies and future directions for systemic lupus erythematosus in adults. Lancet. 2019;393(10188):2332-43. doi:10.1016/s0140-6736(19)30237-5
41. Touma Z, Gladman DD. Current and future therapies for SLE: obstacles and recommendations for the development of novel treatments. Lupus Sci Med. 2017;4(1):e000239. doi:10.1136/lupus-2017-000239
42. Liossis SN, Staveri C. What's New in the Treatment of Systemic Lupus Erythematosus. Front Med. 2021;8:655100. doi:10.3389/fmed.2021.655100
43. Durcan L, Petri M. Why Targeted Therapies are Necessary for Systemic Lupus Erythematosus. Lupus. 2016;25(10):1070-9. doi:10.1177/0961203316652489
44. Vukelic M, Li Y, Kyttaris VC. Novel Treatments in Lupus. Front Immunol. 2018;9:2658. doi:10.3389/fimmu.2018.02658
45. Sciascia S, Radin M, Roccatello D, Sanna G, Bertolaccini. Recent advances in the management of systemic lupus erythematosus. F1000Res. 2018;7: F1000 Faculty Rev-970. doi:10.12688/f1000research.13941.1
2. Chen X, Sun X, Wang W, Yang B, Zhao X, Chen S, et al. An autoimmune disease variant of IgG1 modulates B cell activation and differentiation. Science. 2018;362(6415):700-5. doi:10.1126/science.aap9310
3. Tanaka Y, Mizukami A, Kobayashi A, Ito C, Matsuki T. Disease severity and economic burden in Japanese patients with systemic lupus erythematosus: A retrospective, observational study. Int J Rheum Dis. 2018;21(8):1609-18. doi:10.1111/1756-185X.13363
4. Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of Systemic Lupus Erythematosus. Maedica. 2011;6(4):330-6.
5. Parks CG, Santos AdSE, Barbhaiya M, Costenbader KH. Understanding the role of environmental factors in the development of Systemic Lupus Erythematosus. Best Pract Res Clin Rheumatol. 2017;31(3):306-20. doi:10.1016/j.berh.2017.09.005
6. Constantin MM, Nita IE, Olteanu R, Constantin T, Bucur S, Matei C, et al. Significance and impact of dietary factors on systemic lupus erythematosus pathogenesis. Exp Ther Med. 2019;17(2):1085-90. doi:10.3892/etm.2018.6986
7. Smith CD, Cyr M. The history of lupus erythematosus. From Hippocrates to Osler. Rheum Dis Clin North Am. 1988;14(1):1-14.
8. Boumpas DT, Bertsias GK, Fanouriakis A. 2008-2018: a decade of recommendations for systemic lupus erythematosus. Ann Rheum Dis. 2018;77(11):1547-8. doi:10.1136/annrheumdis-2018-214014
9. Bae EH, Lim SY, Han KD, Jung JH, Choi HS, Kim HY, et al. Trend of prevalence and incidence of systemic lupus erythematosus in South Korea, 2005 to 2015: a nationwide population-based study. Korean J Intern Med. 2020;35(3):652-61. doi:10.3904/kjim.2018.303
10. Fava A, Petri M. Systemic Lupus Erythematosus: Diagnosis and Clinical Management. J Autoimmun. 2019;96:1-13. doi:10.1016/j.jaut.2018.11.001
11. Stojan G, Petri M. Epidemiology of Systemic Lupus Erythematosus: an update. Curr Opin Rheumatol. 2018;30(2):144-50. doi:10.1097/BOR.0000000000000480
12. Fatoye F, Gebrye T, Svenson LW. Real-world incidence and prevalence of systemic lupus erythematosus in Alberta, Canada. Rheumatol Int. 2018;38(9):1721-6. doi:10.1007/s00296-018-4091-4
13. Bharath G, Kumar P, Makkar N, Singla P, Soneja M, Biswas A, et al. Mortality in systemic lupus erythematosus at a teaching hospital in India: A 5-year retrospective study. J Family Med Prim Care. 2019;8(7):2511-5. doi:10.4103/jfmpc.jfmpc_362_19
14. Osio-Salido E, Manapat-Reyes H. Epidemiology of systemic lupus erythematosus in Asia. Lupus. 2010;19(12):1365-73. doi:10.1177/0961203310374305
15. Gergianaki I, Bertsias G. Systemic Lupus Erythematosus in Primary Care: An Update and Practical Messages for the General Practitioner. Front Med. 2018;5:161. doi:10.3389/fmed.2018.00161
16. Williams EM, Bruner L, Adkins A, Vrana C, Logan A, Kamen D, et al. I too, am America: a review of research on systemic lupus erythematosus in African-Americans. Lupus Sci Med. 2016;3(1):e000144. doi:10.1136/lupus-2015-000144
17. Olesińska M, Saletra A. Quality of life in systemic lupus erythematosus and its measurement. Reumatologia. 2018;56(1):45-54. doi:10.5114/reum.2018.74750
18. Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices. Kidney Int. 2018;93(4):789-96. doi:10.1016/j.kint.2017.11.023
19. Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736-45. doi:10.1136/annrheumdis-2019-215089
20. Hon KL, Leung AKC. Neonatal Lupus Erythematosus. Autoimmune Dis. 2012;2012:301274. doi:10.1155/2012/301274
21. Costagliola G, Mosca M, Migliorini P, Consolini R. Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood. Front Pediatr. 2018;6:144. doi:10.3389/fped.2018.00144
22. Okon LG, Werth VP. Cutaneous Lupus Erythematosus: Diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27(3):391-404. doi:10.1016/j.berh.2013.07.008
23. Sarzi-Puttini P, Atzeni F, Capsoni F, Lubrano E, Doria A. Drug-induced lupus erythematosus. Autoimmunity. 2005;38(7):507-18. doi:10.1080/08916930500285857
24. Rekvig OP. Systemic Lupus Erythematosus: Definitions, Contexts, Conflicts, Enigmas. Front Immunol. 2018;9:387. doi:10.3389/fimmu.2018.00387
25. Moulton VR, Suarez-Fueyo A, Meidan E, Li H, Mizui M, Tsokos GC. Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective. Trend Mol Med. 2017;23(7):615-35. doi:10.1016/j.molmed.2017.05.006
26. Barbhaiya M, Costenbader KH. Environmental Exposures and the Development of Systemic Lupus Erythematosus. Curr Opin Rheumatol. 2016;28(5):497-505. doi:10.1097/BOR.0000000000000318
27. Arneth B. Systemic Lupus Erythematosus and DNA Degradation and Elimination Defects. Front Immunol. 2019;10:1697. doi:10.3389/fimmu.2019.01697
28. Zharkova O, Celhar T, Cravens PD, Satterthwaite AB, Fairhurst AM, Davis LS. Pathways leading to an immunological disease: systemic lupus erythematosus. Rheumatology. 2017;56(suppl_1):i55-i66. doi:10.1093/rheumatology/kew427
29. Pan Q, Chen J, Guo L, Lu X, Liao S, Zhao C, et al. Mechanistic insights into environmental and genetic risk factors for systemic lupus erythematosus. Am J Transl Res. 2019;11(3):1241-54.
30. Pan L, Lu MP, Wang JH, Xu M, Yang SR. Immunological pathogenesis and treatment of systemic lupus erythematosus. World J Pediatr. 2020;16(1):19-30. doi:10.1007/s12519-019-00229-3
31. Liao X, Reihl AM, Luo XM. Breakdown of Immune Tolerance in Systemic Lupus Erythematosus by Dendritic Cells. J Immunol Res. 2016;2016:6269157. doi:10.1155/2016/6269157
32. Avasare RS, Yee J. Lupus Nephritis: Breaking the Lull. Adv Chronic Kidney Dis. 2019;26(5):307-10. doi:10.1053/j.ackd.2019.05.005
33. Kuhn A, Bonsmann G, Anders HJ, Herzer P, Tenbrock K, Schneider M. The Diagnosis and Treatment of Systemic Lupus Erythematosus. Dtsch Arztebl Int. 2015;112(25):423-32. doi:10.3238/arztebl.2015.0423
34. Aringer M, Leuchten N, Johnson SR. New Criteria for Lupus. Curr Rheumatol Rep. 2020;22(6):18. doi:10.1007/s11926-020-00896-6
35. Vasquez-Canizares N, Wahezi D, Putterman C. Diagnostic and Prognostic Tests in Systemic Lupus Erythematosus. Best Pract Res Clin Rheumatol. 2017;31(3):351-63. doi:10.1016/j.berh.2017.10.002
36. Adamichou C, Bertsias G. Flares in systemic lupus erythematosus: diagnosis, risk factors and preventive strategies. Mediterr J Rheumatol. 2017;28(1):4-12. doi:10.31138/mjr.28.1.4
37. Lam NCV, Ghetu MV, Bieniek ML. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician. 2016;94(4):284-94.
38. Basta F, Fasola F, Triantafyllias K, Schwarting A. Systemic Lupus Erythematosus (SLE) Therapy: The Old and the New. Rheumatol Ther. 2020;7(3):433-46. doi:10.1007/s40744-020-00212-9
39. Ruiz-Irastorza G, Bertsias G. Treating systemic lupus erythematosus in the 21st century: new drugs and new perspectives on old drugs. Rheumatology. 2020;59(Suppl 5):v69-v81. doi:10.1093/rheumatology/keaa403
40. Durcan L, O’Dwyer T, Petri M. Management strategies and future directions for systemic lupus erythematosus in adults. Lancet. 2019;393(10188):2332-43. doi:10.1016/s0140-6736(19)30237-5
41. Touma Z, Gladman DD. Current and future therapies for SLE: obstacles and recommendations for the development of novel treatments. Lupus Sci Med. 2017;4(1):e000239. doi:10.1136/lupus-2017-000239
42. Liossis SN, Staveri C. What's New in the Treatment of Systemic Lupus Erythematosus. Front Med. 2021;8:655100. doi:10.3389/fmed.2021.655100
43. Durcan L, Petri M. Why Targeted Therapies are Necessary for Systemic Lupus Erythematosus. Lupus. 2016;25(10):1070-9. doi:10.1177/0961203316652489
44. Vukelic M, Li Y, Kyttaris VC. Novel Treatments in Lupus. Front Immunol. 2018;9:2658. doi:10.3389/fimmu.2018.02658
45. Sciascia S, Radin M, Roccatello D, Sanna G, Bertolaccini. Recent advances in the management of systemic lupus erythematosus. F1000Res. 2018;7: F1000 Faculty Rev-970. doi:10.12688/f1000research.13941.1
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Nimesh S, Ahmad MI, Dhama S, Kumar P, Akram M, Hasaroeih NEN. Systemic Lupus Erythematosus Disease: An Overview of the Clinical Approach to Pathogenesis, Diagnosis, and Treatment. Borneo J Pharm [Internet]. 2021May30 [cited 2024Apr.19];4(2):91-8. Available from: https://journal.umpr.ac.id/index.php/bjop/article/view/1950
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