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Mortality in patients with systemic lupus erythematosus in Colombia: a case series

  • David Aguirre-Valencia
  • Ana Suárez-Avellaneda
  • Vanessa Ocampo-Piraquive
  • Iván Posso-Osorio
  • Juan Naranjo-Escobar
  • Ivana Nieto-Aristizábal
  • Gabriel J. TobónEmail author
  • Carlos A. Cañas
Original Article

Abstract

Introduction

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease associated with high mortality rates. This study aimed to describe the main causes of death in a case series of SLE patients attended in a single center in Colombia.

Methods

We conducted a retrospective review and analysis of records of SLE patients who died between January 2011 and June 2017. We extracted the main causes of death and described variables associated with this outcome as well as variables associated with the disease and its treatment.

Results

From a total of 1776 patients with SLE, we identified 49 fatal cases (89.8% women, n = 44). The average age at death was 40.6 years (SD 17.4), and patients had a median of 4.5 years (IQR 2–8) of disease duration. The main findings included lymphopenia in 44 patients (89.9%), biopsy-confirmed lupus nephritis (LN)—types IV and VI—in 38 (77.6%), catastrophic antiphospholipid syndrome (CAPS) in 8 (16.3%), and persistent hypocomplementemia (C3 and C4) in 8 (16.3%). The median SLE disease activity index (SLEDAI-2K) score at the time of death was 19 (IQR 11–39). The main cause of death was SLE activity and lupus-induced damage in 22 (44.9%) patients.

Conclusion

The main causes of death included SLE activity refractory to immunosuppressive treatment, and nosocomial bacterial infections. The patients who died had persistently high SLEDAI scores, types IV and VI LN, associated antiphospholipid syndrome, and persistent hypocomplementemia, requiring severe immunosuppression and prolonged hospitalization.

Keywords

Immunosuppression Mortality Outcomes SLEDAI Systemic lupus erythematosus 

Notes

Compliance with ethical standards

Disclosures

None.

References

  1. 1.
    Bernatsky S, Boivin J-F, Joseph L, Manzi S, Ginzler E, Gladman DD, Urowitz M, Fortin PR, Petri M, Barr S, Gordon C, Bae SC, Isenberg D, Zoma A, Aranow C, Dooley MA, Nived O, Sturfelt G, Steinsson K, Alarcón G, Senécal JL, Zummer M, Hanly J, Ensworth S, Pope J, Edworthy S, Rahman A, Sibley J, el-Gabalawy H, McCarthy T, St. Pierre Y, Clarke A, Ramsey-Goldman R (2006) Mortality in systemic lupus erythematosus. Arthritis Rheum 54:2550–2557.  https://doi.org/10.1002/art.21955 CrossRefGoogle Scholar
  2. 2.
    Kasitanon N, Magder LS, Petri M (2006) Predictors of survival in systemic lupus erythematosus. Medicine (Baltimore) 85:147–156.  https://doi.org/10.1097/01.md.0000224709.70133.f7 CrossRefGoogle Scholar
  3. 3.
    Fors Nieves CE, Izmirly PM (2016) Mortality in systemic lupus erythematosus: an updated review. Curr Rheumatol Rep 18(21):21.  https://doi.org/10.1007/s11926-016-0571-2 CrossRefGoogle Scholar
  4. 4.
    Pons-Estel GJ, Ugarte-Gil MF, Alarcón GS (2017) Epidemiology of systemic lupus erythematosus. Expert Rev Clin Immunol 13:799–814.  https://doi.org/10.1080/1744666X.2017.1327352 CrossRefGoogle Scholar
  5. 5.
    Danila MI, Pons-Estel GJ, Zhang J, Vila LM, Reveille JD, Alarcon GS (2008) Renal damage is the most important predictor of mortality within the damage index: data from LUMINA LXIV, a multiethnic US cohort. Rheumatology 48:542–545.  https://doi.org/10.1093/rheumatology/kep012 CrossRefGoogle Scholar
  6. 6.
    Andrade RM, Alarcón GS, Fernández M, Apte M, Vilá LM, Reveille JD, LUMINA Study Group (2007) Accelerated damage accrual among men with systemic lupus erythematosus: XLIV. Results from a multiethnic US cohort. Arthritis Rheum 56:622–630.  https://doi.org/10.1002/art.22375 CrossRefGoogle Scholar
  7. 7.
    Lee YH, Choi SJ, Ji JD, Song GG (2016) Overall and cause-specific mortality in systemic lupus erythematosus: an updated meta-analysis. Lupus 25:727–734.  https://doi.org/10.1177/0961203315627202 CrossRefGoogle Scholar
  8. 8.
    Pons-Estel GJ, Catoggio LJ, Cardiel MH, Bonfa E, Caeiro F, Sato E, Massardo L, Molina-Restrepo JF, Toledano MG, Barile-Fabris LA, Amigo MC, Acevedo-Vásquez EM, Abadi I, Wojdyla D, Alarcón-Riquelme ME, Alarcón GS, Pons-Estel BA (2015) Lupus in Latin-American patients: lessons from the GLADEL cohort. Lupus 24:536–545.  https://doi.org/10.1177/0961203314567753 CrossRefGoogle Scholar
  9. 9.
    Legge A, Kirkland S, Rockwood K, Andreou P, Bae SC, Gordon C, Romero-Diaz J, Sanchez-Guerrero J, Wallace DJ, Bernatsky S, Clarke AE, Merrill JT, Ginzler EM, Fortin P, Gladman DD, Urowitz MB, Bruce IN, Isenberg DA, Rahman A, Alarcón GS, Petri M, Khamashta MA, Dooley MA, Ramsey-Goldman R, Manzi S, Steinsson K, Zoma AA, Aranow C, Mackay M, Ruiz-Irastorza G, Lim SS, Inanc M, van Vollenhoven RF, Jonsen A, Nived O, Ramos-Casals M, Kamen DL, Kalunian KC, Jacobsen S, Peschken CA, Askanase A, Hanly JG (2019) Evaluating the properties of a frailty index and its association with mortality risk among patients with systemic lupus erythematosus. Arthritis Rheum:0–3.  https://doi.org/10.1002/art.40859
  10. 10.
    Rees F, Doherty M, Grainge MJ, Lanyon P, Davenport G, Zhang W (2016) Mortality in systemic lupus erythematosus in the United Kingdom 1999–2012. Rheumatology 55:854–860.  https://doi.org/10.1093/rheumatology/kev424 CrossRefGoogle Scholar
  11. 11.
    Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725.  https://doi.org/10.1002/1529-0131(199709)40:9<1725::AID-ART29>3.0.CO;2-Y CrossRefGoogle Scholar
  12. 12.
    Merola J, Bermas B, Lu B, Karlson EW, Massarotti E, Schur PH, Costenbader KH (2014) Clinical manifestations and survival among adults with (SLE) according to age at diagnosis. Lupus 23:778–784.  https://doi.org/10.1177/0961203314526291 CrossRefGoogle Scholar
  13. 13.
    Costi LR, Iwamoto HM, Neves DC de O, Caldas CAM (2017) Mortality from systemic erythematosus lupus in Brazil: evaluation of causes according to the government health database. Rev Bras Reumatol Engl Ed 57:574–582.  https://doi.org/10.1016/J.RBRE.2017.09.001 CrossRefGoogle Scholar
  14. 14.
    Bruce IN, O’Keeffe AG, Farewell V et al (2015) Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort. Ann Rheum Dis 74:1706–1713.  https://doi.org/10.1136/annrheumdis-2013-205171 CrossRefGoogle Scholar
  15. 15.
    Gómez-Puerta JA, Barbhaiya M, Guan H, Feldman CH, Alarcón GS, Costenbader KH (2015) Racial/ethnic variation in all-cause mortality among United States Medicaid recipients with systemic lupus erythematosus: a Hispanic and Asian paradox. Arthritis Rheum 67:752–760.  https://doi.org/10.1002/art.38981 CrossRefGoogle Scholar
  16. 16.
    Almaani S, Meara A, Rovin BH (2017) Update on lupus nephritis. Clin J Am Soc Nephrol 12:825–835.  https://doi.org/10.2215/CJN.05780616 CrossRefGoogle Scholar
  17. 17.
    Feldman CH, Hiraki LT, Winkelmayer WC, Marty FM, Franklin JM, Kim SC, Costenbader KH (2015) Serious infections among adult Medicaid beneficiaries with systemic lupus erythematosus and lupus nephritis. Arthritis Rheum 67:1577–1585.  https://doi.org/10.1002/art.39070 CrossRefGoogle Scholar
  18. 18.
    Ichinose K, Kitamura M, Sato S, Fujikawa K, Horai Y, Matsuoka N, Tsuboi M, Nonaka F, Shimizu T, Fukui S, Umeda M, Koga T, Kawashiri S, Iwamoto N, Tamai M, Nakamura H, Origuchi T, Nishino T, Kawakami A (2019) Factors predictive of long-term mortality in lupus nephritis: a multicenter retrospective study of a Japanese cohort. Lupus 28:295–303.  https://doi.org/10.1177/0961203319826690 CrossRefGoogle Scholar
  19. 19.
    Agarwal S, Elliott JR, Manzi S (2009) Atherosclerosis risk factors in systemic lupus erythematosus. Curr Rheumatol Rep 11:241–247CrossRefGoogle Scholar
  20. 20.
    Turesson C, O’Fallon WM, Crowson CS et al (2003) Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and risk factors over 46 years. Ann Rheum Dis 62:722–727.  https://doi.org/10.1136/ARD.62.8.722 CrossRefGoogle Scholar
  21. 21.
    Rodríguez-Pintó I, Moitinho M, Santacreu I, Shoenfeld Y, Erkan D, Espinosa G, Cervera R, CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies) (2016) Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev 15:1120–1124.  https://doi.org/10.1016/J.AUTREV.2016.09.010 CrossRefGoogle Scholar
  22. 22.
    Ocampo-Piraquive V, Nieto-Aristizábal I, Cañas CA, Tobón GJ (2018) Mortality in systemic lupus erythematosus: causes, predictors and interventions. Expert Rev Clin Immunol 14:1043–1053.  https://doi.org/10.1080/1744666X.2018.1538789 CrossRefGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  • David Aguirre-Valencia
    • 1
    • 2
  • Ana Suárez-Avellaneda
    • 2
  • Vanessa Ocampo-Piraquive
    • 2
  • Iván Posso-Osorio
    • 1
  • Juan Naranjo-Escobar
    • 1
  • Ivana Nieto-Aristizábal
    • 1
  • Gabriel J. Tobón
    • 1
    • 2
    • 3
    Email author
  • Carlos A. Cañas
    • 1
    • 2
  1. 1.GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina TraslacionalFundación Valle del Lili and Universidad IcesiCaliColombia
  2. 2.Medical SchoolUniversidad IcesiCaliColombia
  3. 3.Immunology LaboratoryFundación Valle del LiliCaliColombia

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