Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Current Rheumatology Reports
Published in: Current Rheumatology Reports 4/2016

01-04-2016 | Systemic Lupus Erythematosus (G Tsokos, Guest Section Editor)

Mortality in Systemic Lupus Erythematosus: an Updated Review

Authors: César E. Fors Nieves, Peter M. Izmirly

Published in: Current Rheumatology Reports | Issue 4/2016

Login to get access

Abstract

Both systemic lupus erythematosus (SLE) and its treatments can contribute to increased mortality rates. The focus of this review is recent studies on mortality and comorbidities during the last 5 years from around the world. The authors conducted a literature review, using PUBMED, for articles relating to SLE mortality with a specific focus on literature published within the last 5 years. Our analysis found that while mortality in SLE patients continues to improve, there are differences in survival based on ethnicity, socioeconomic status, age, and gender. The most common cause of mortality is cardiovascular disease, followed closely by infection and severe disease activity. To conclude, while there have been significant advances in the treatment of SLE and its associated comorbidities, increased mortality remains a major concern in patient management.
Literature
1.
Bernatsky S, Boivin JF, Joseph L, Manzi S, Ginzler E, Gladman DD, et al. Mortality in systemic lupus erythematosus. Arthritis Rheum. 2006;54(8):2550–7.CrossRefPubMed
2.
Kasitanon N, Magder LS, Petri M. Predictors of survival in systemic lupus erythematosus. Medicine (Baltimore). 2006;85(3):147–56.CrossRef
3.
Doria A, Iaccarino L, Ghirardello A, Zampieri S, Arienti S, Sarzi-Puttini P, et al. Long-term prognosis and causes of death in systemic lupus erythematosus. Am J Med. 2006;119(8):700–6.CrossRefPubMed
4.
Prasad R, Ibanez D, Gladman D, Urowitz M. Anti-dsDNA and anti-Sm antibodies do not predict damage in systemic lupus erythematosus. Lupus. 2006;15(5):285–91.CrossRefPubMed
5.
Andrade RM, Alarcon GS, Fernandez M, Apte M, Vila LM, Reveille JD, et al. Accelerated damage accrual among men with systemic lupus erythematosus: XLIV. Results from a multiethnic U.S. cohort. Arthritis Rheum. 2007;56(2):622–30.CrossRefPubMed
6.
Mayor AM, Vila LM. Gender differences in a cohort of Puerto Ricans with systemic lupus erythematosus. Cell Mol Biol (Noisy-le-Grand). 2003;49(8):1339–44.
7.
Bujan S, Ordi-Ros J, Paredes J, Mauri M, Matas L, Cortes J, et al. Contribution of the initial features of systemic lupus erythematosus to the clinical evolution and survival of a cohort of Mediterranean patients. Ann Rheum Dis. 2003;62(9):859–65.CrossRefPubMedPubMedCentral
8.
Hersh AO, Trupin L, Yazdany J, Panopalis P, Julian L, Katz P, et al. Childhood-onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2010;62(8):1152–9.CrossRef
9.
Urowitz MB, Gladman DD, Tom BD, Ibanez D, Farewell VT. Changing patterns in mortality and disease outcomes for patients with systemic lupus erythematosus. J Rheumatol. 2008;35(11):2152–8.CrossRefPubMed
10.
Merrell M, Shulman LE. Determination of prognosis in chronic disease, illustrated by systemic lupus erythematosus. J Chronic Dis. 1955;1(1):12–32.CrossRefPubMed
11.
Abu-Shakra M, Gladman DD, Urowitz MB. Mortality studies in SLE: how far can we improve survival of patients with SLE. Autoimmun Rev. 2004;3(6):418–20.CrossRefPubMed
12.
Urowitz MB, Gladman DD. How to improve morbidity and mortality in systemic lupus erythematosus. Rheumatology (Oxford). 2000;39(3):238–44.CrossRef
13.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, 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.CrossRef
14.
Bellomio V, Spindler A, Lucero E, Berman A, Santana M, Moreno C, et al. Systemic lupus erythematosus: mortality and survival in Argentina. A multicenter study. Lupus. 2000;9(5):377–81.PubMed
15.
Alamanos Y, Voulgari PV, Siozos C, Katsimpri P, Tsintzos S, Dimou G, et al. Epidemiology of systemic lupus erythematosus in northwest Greece 1982-2001. J Rheumatol. 2003;30(4):731–5.PubMed
16.
Mok CC, Mak A, Chu WP, To CH, Wong SN. Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups. Medicine (Baltimore). 2005;84(4):218–24.CrossRef
17.
Sun HO, Hu WX, Xie HL, Zhang HT, Chen HP, Zeng CH, et al. Long-term outcome of Chinese patients with membranous lupus nephropathy. Lupus. 2008;17(1):56–61.CrossRefPubMed
18.
Al Saleh J, El Sayed M, Jassim V, Mohammed N, Khan N. Hypothyroidism determines the clinical and immunological manifestations of Arabs with lupus. Lupus. 2008;17(3):215–20.CrossRefPubMed
19.
Funauchi M, Shimadzu H, Tamaki C, Yamagata T, Nozaki Y, Sugiyama M, et al. Survival study by organ disorders in 306 Japanese patients with systemic lupus erythematosus: results from a single center. Rheumatol Int. 2007;27(3):243–9.CrossRefPubMed
20.
21.••
Gomez-Puerta JA, Barbhaiya M, Guan H, Feldman CH, Alarcon GS, Costenbader KH. Racial/Ethnic variation in all-cause mortality among United States medicaid recipients with systemic lupus erythematosus: a Hispanic and asian paradox. Arthritis Rheum. 2015;67(3):752–60. One of the largest, if not the largest study on mortality in SLE published from the United States. The authors reported a variation in mortality rates among Medicaid patients with SLE according to race/ethnicity. In particular they reported a lower mortality rate among Asian and Hispanic patients when compared to black, white, or Native American patients. CrossRef
22.
Bartels CM, Buhr KA, Goldberg JW, Bell CL, Visekruna M, Nekkanti S, et al. Mortality and cardiovascular burden of systemic lupus erythematosus in a U.S. population-based cohort. J Rheumatol. 2014;41(4):680–7.CrossRefPubMedPubMedCentral
23.
Merola JF, Bermas B, Lu B, Karlson EW, Massarotti E, Schur PH, et al. Clinical manifestations and survival among adults with (SLE) according to age at diagnosis. Lupus. 2014;23(8):778–84.CrossRefPubMedPubMedCentral
24.
Sule S, Fivush B, Neu A, Furth S. Increased risk of death in pediatric and adult patients with ESRD secondary to lupus. Pediatr Nephrol. 2011;26(1):93–8.CrossRefPubMedPubMedCentral
25.
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 U.S. cohort. Rheumatology (Oxford). 2009;48(5):542–5.CrossRef
26.•
Ruiz E, Ramalle-Gomara E, Elena A, Quinones C, Alonso V, Posada M, et al. Trends in systemic lupus erythematosus mortality in Spain from 1981 to 2010. Lupus. 2014;23(4):431–5. Interesting study from Spain showing decreasing mortality over the last decade. CrossRefPubMed
27.
Thomas G, Mancini J, Jourde-Chiche N, Sarlon G, Amoura Z, Harle JR, et al. Mortality associated with systemic lupus erythematosus in France assessed by multiple cause-of-death analysis: the MORTALUP Study. Arthritis Rheum. 2014. doi:10.​1002/​art.​38731.
28.
Elfving P, Puolakka K, Kautiainen H, Virta L, Pohjolainen T, Kaipiainen-Seppanen O. Mortality and causes of death among incident cases of systemic lupus erythematosus in Finland 2000-2008. Lupus. 2014. doi:10.​1177/​0961203314543919​.PubMed
29.•
Lerang K, Gilboe IM, Steinar Thelle D, Gran JT. Mortality and years of potential life loss in systemic lupus erythematosus: a population-based cohort study. Lupus. 2014;23(14):1546–52. Scandinavian study showing current mortality rates in a relatively homogeneous Northern European cohort. CrossRefPubMed
30.
Faurschou M, Dreyer L, Kamper AL, Starklint H, Jacobsen S. Long-term mortality and renal outcome in a cohort of 100 patients with lupus nephritis. Arthritis Care Res (Hoboken). 2010;62(6):873–80.CrossRef
31.
Yang, Y., Thumboo, J., Earnest, A., Yong, S., & Fong, K. (2014). The effect of comorbidity on hospital mortality in patients with SLE from an Asian tertiary hospital. Lupus.
32.
Chen YM, Lin CH, Chen HH, Chang SN, Hsieh TY, Hung WT, et al. Onset age affects mortality and renal outcome of female systemic lupus erythematosus patients: a nationwide population-based study in Taiwan. Rheumatology (Oxford). 2014;53(1):180–5.CrossRef
33.••
Mok CC, Kwok RC, Yip PS. Effect of renal disease on the standardized mortality ratio and life expectancy of patients with systemic lupus erythematosus. Arthritis Rheum. 2013;65(8):2154–60. Comprehensive, well done study from Hong Kong linking renal insufficiency with reduced survival in an all Chinese SLE cohort. CrossRefPubMed
34.
Lin WH, Guo CY, Wang WM, Yang DC, Kuo TH, Liu MF, et al. Incidence of progression from newly diagnosed systemic lupus erythematosus to end stage renal disease and all-cause mortality: a nationwide cohort study in Taiwan. Int J Rheum Dis. 2013;16(6):747–53.CrossRefPubMed
35.•
Flower C, Hennis AJ, Hambleton IR, Nicholson GD, Liang MH, Barbados National Lupus Registry, G. Systemic lupus erythematosus in an African Caribbean population: incidence, clinical manifestations, and survival in the Barbados National Lupus Registry. Arthritis Care Res (Hoboken). 2012;64(8):1151–8. Important study given the demographics of the cohort. The authors looked at survival and risk factors for increased mortality in a cohort made up mainly (98%) of African Caribbean patients of West African descent. They reported a very high incidence rate of SLE in these patients and an overall lower survival rates at 5 years than in the U.S. based cohorts.
36.
Al Arfaj AS, Khalil N, Al Saleh S. Lupus nephritis among 624 cases of systemic lupus erythematosus in Riyadh, Saudi Arabia. Rheumatol Int. 2009;29(9):1057–67.CrossRefPubMed
37.
Yap DY, Tang CS, Ma MK, Lam MF, Chan TM. Survival analysis and causes of mortality in patients with lupus nephritis. Nephrol Dial Transplant. 2012;27(8):3248–54.CrossRefPubMed
38.
Zonana-Nacach A, Yanez P, Jimenez-Balderas FJ, Camargo-Coronel A. Disease activity, damage and survival in Mexican patients with acute severe systemic lupus erythematosus. Lupus. 2007;16(12):997–1000.CrossRefPubMed
39.
Ward MM, Pyun E, Studenski S. Mortality risks associated with specific clinical manifestations of systemic lupus erythematosus. Arch Intern Med. 1996;156(12):1337–44.CrossRefPubMed
40.
Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus. Results from a single center. J Rheumatol. 1995;22(7):1259–64.PubMed
41.
Rubin LA, Urowitz MB, Gladman DD. Mortality in systemic lupus erythematosus: the bimodal pattern revisited. Q J Med. 1985;55(216):87–98.PubMed
42.
Moss KE, Ioannou Y, Sultan SM, Haq I, Isenberg DA. Outcome of a cohort of 300 patients with systemic lupus erythematosus attending a dedicated clinic for over two decades. Ann Rheum Dis. 2002;61(5):409–13.CrossRefPubMedPubMedCentral
43.
Bjornadal L, Yin L, Granath F, Klareskog L, Ekbom A. Cardiovascular disease a hazard despite improved prognosis in patients with systemic lupus erythematosus: results from a Swedish population based study 1964-95. J Rheumatol. 2004;31(4):713–9.PubMed
44.
Cartella S, Cavazzana I, Ceribelli A, Inverardi F, Tincani A, Franceschini F. Evaluation of mortality, disease activity, treatment, clinical and immunological features of adult and late onset systemic Lupus erythematosus. Autoimmunity. 2013;46(6):363–8.CrossRefPubMed
45.••
Yurkovich M, Vostretsova K, Chen W, Avina-Zubieta JA. Overall and cause-specific mortality in patients with systemic lupus erythematosus: a meta-analysis of observational studies. Arthritis Care Res (Hoboken). 2014;66(4):608–16. Large scale meta-analysis looking at mortality rates in lupus and risk factors for increased mortality. CrossRef
46.
Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH, et al. Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia. J Natl Cancer Inst. 2006;98(18):1321–30.CrossRefPubMed
47.
Mellemkjaer L, Pfeiffer RM, Engels EA, Gridley G, Wheeler W, Hemminki K, et al. Autoimmune disease in individuals and close family members and susceptibility to non-Hodgkin’s lymphoma. Arthritis Rheum. 2008;58(3):657–66.CrossRefPubMed
48.
Bernatsky S, Ramsey-Goldman R, Isenberg D, Rahman A, Dooley MA, Sibley J, et al. Hodgkin's lymphoma in systemic lupus erythematosus. Rheumatology (Oxford). 2007;46(5):830–2.CrossRef
49.
Bulkley BH, Roberts WC. The heart in systemic lupus erythematosus and the changes induced in it by corticosteroid therapy. A study of 36 necropsy patients. Am J Med. 1975;58(2):243–64.CrossRefPubMed
50.
Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger Jr TA, Jansen-McWilliams L, et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol. 1997;145(5):408–15.CrossRefPubMed
51.
Urowitz MB, Bookman AA, Koehler BE, Gordon DA, Smythe HA, Ogryzlo MA. The bimodal mortality pattern of systemic lupus erythematosus. Am J Med. 1976;60(2):221–5.CrossRefPubMed
52.
Asanuma Y, Oeser A, Shintani AK, Turner E, Olsen N, Fazio S, et al. Premature coronary-artery atherosclerosis in systemic lupus erythematosus. N Engl J Med. 2003;349(25):2407–15.CrossRefPubMed
53.
Roman MJ, Shanker BA, Davis A, Lockshin MD, Sammaritano L, Simantov R, et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med. 2003;349(25):2399–406.CrossRefPubMed
54.
Agarwal S, Elliott JR, Manzi S. Atherosclerosis risk factors in systemic lupus erythematosus. Curr Rheumatol Rep. 2009;11(4):241–7.CrossRefPubMed
55.
Packard RR, Libby P. Inflammation in atherosclerosis: from vascular biology to biomarker discovery and risk prediction. Clin Chem. 2008;54(1):24–38.CrossRefPubMed
56.
Hansson GK. Inflammation, atherosclerosis, and coronary artery disease. N Engl J Med. 2005;352(16):1685–95.CrossRefPubMed
57.
58.
Hahn BH, McMahon M. Atherosclerosis and systemic lupus erythematosus: the role of altered lipids and of autoantibodies. Lupus. 2008;17(5):368–70.CrossRefPubMed
59.
Borba EF, Bonfa E. Dyslipoproteinemias in systemic lupus erythematosus: influence of disease, activity, and anticardiolipin antibodies. Lupus. 1997;6(6):533–9.CrossRefPubMed
60.
Petri M. Detection of coronary artery disease and the role of traditional risk factors in the Hopkins Lupus Cohort. Lupus. 2000;9(3):170–5.CrossRefPubMed
61.
Borba EF, Santos RD, Bonfa E, Vinagre CG, Pileggi FJ, Cossermelli W, et al. Lipoprotein(a) levels in systemic lupus erythematosus. J Rheumatol. 1994;21(2):220–3.PubMed
62.
Shoenfeld Y, Wu R, Dearing LD, Matsuura E. Are anti-oxidized low-density lipoprotein antibodies pathogenic or protective? Circulation. 2004;110(17):2552–8.CrossRefPubMed
63.
de Leeuw K, Freire B, Smit AJ, Bootsma H, Kallenberg CG, Bijl M. Traditional and non-traditional risk factors contribute to the development of accelerated atherosclerosis in patients with systemic lupus erythematosus. Lupus. 2006;15(10):675–82.CrossRefPubMed
64.••
Feldman CH, Hiraki LT, Winkelmayer WC, Marty FM, Franklin JM, Kim SC, et al. Serious infections among adult Medicaid beneficiaries with systemic lupus erythematosus and lupus nephritis. Arthritis Rheum. 2015;67(6):1577–85. Another large scale study using the U.S. based Medicaid database with over 33,000 patients of various races/ethnicities, delineates the burden of serious infections in patients with SLE, and shows a particularly higher risk of mortality among patients with LN and infection. CrossRef
65.
Borchers AT, Keen CL, Shoenfeld Y, Gershwin ME. Surviving the butterfly and the wolf: mortality trends in systemic lupus erythematosus. Autoimmun Rev. 2004;3(6):423–53.CrossRefPubMed
66.
Nossent J, Cikes N, Kiss E, Marchesoni A, Nassonova V, Mosca M, et al. Current causes of death in systemic lupus erythematosus in Europe, 2000–2004: relation to disease activity and damage accrual. Lupus. 2007;16(5):309–17.CrossRefPubMed
67.
Wadee S, Tikly M, Hopley M. Causes and predictors of death in South Africans with systemic lupus erythematosus. Rheumatology (Oxford). 2007;46(9):1487–91.CrossRef
68.
Ward MM, Pajevic S, Dreyfuss J, Malley JD. Short-term prediction of mortality in patients with systemic lupus erythematosus: classification of outcomes using random forests. Arthritis Rheum. 2006;55(1):74–80.CrossRefPubMed
Metadata
Title
Mortality in Systemic Lupus Erythematosus: an Updated Review
Authors
César E. Fors Nieves
Peter M. Izmirly
Publication date
01-04-2016
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 4/2016
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-016-0571-2

Other articles of this Issue 4/2016

Current Rheumatology Reports 4/2016 Go to the issue

Chronic Pain (R Staud, Section Editor)

Juvenile Fibromyalgia: Different from the Adult Chronic Pain Syndrome?

Pediatric Rheumatology (S Ozen, Section Editor)

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis

Antiphospholipid Syndrome (S Zuily, Section Editor)

Emerging Therapies in Antiphospholipid Syndrome

Surgery and Perioperative Care (CR MacKenzie and SM Goodman, Section Editors)

Racial/Ethnic Disparity in Rates and Outcomes of Total Joint Arthroplasty
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine
Image Credits