Skip to main content

Late-Onset Systemic Lupus Erythematosus

Epidemiology, Diagnosis and Treatment

Drugs & Aging volume 29, pages 181–189 (2012)Cite this article

Abstract

Systemic lupus erythematosus (SLE) is usually described as a disease that most often strikes reproductive-age women. However, the onset of SLE beyond the age of 50 years is reported to occur in 3–18% of patients. This later age at onset has a strong modifying effect on the clinical presentation, disease course, response to treatment and prognosis of SLE. In comparison with younger patients, patients with late-onset SLE often have delayed diagnosis and less common occurrence of severe manifestations. For instance, literature data suggest that pulmonary involvement and serositis are more frequent in late-onset SLE, whereas malar rash, photosensitivity, arthritis and nephropathy occur less commonly. Furthermore, some distinct aspects of late-onset SLE may be influenced by the association with Sjögren’s syndrome, which is more frequent in the elderly. Not only clinical features but also serological manifestations of SLE change with aging. In comparison with early-onset SLE patients, older patients have a higher frequency of rheumatoid factor and of antinuclear antibody positivity, and a lower frequency of anti-ribonucleoprotein (anti-RNP) and anti-Sm antibody positivity. The major challenge for physicians managing patients with SLE is to treat the active phase without allowing the treatment itself to cause long-term damage. This is especially true in older and often polymedicated patients, in whom side effects of treatments are more frequent. Another important issue is that possible drug interactions should always be considered in the elderly. The management of the disease in these older patients depends on the type and severity of disease manifestations. The use of antimalarial agents such as hydroxychloroquine is an important aspect of SLE treatment, unless contraindicated. Other treatments mostly include NSAIDs, corticosteroids and immunosuppressive agents, depending on which organs are involved. Survival data may be used as an indirect way to assess the changes in the severity of SLE with aging, but the few studies available conclude that late-onset SLE is associated with poorer survival than early-onset SLE, which likely reflects the consequences of aging rather than true differences in survival. Importantly, the cause of death in late-onset SLE patients is usually not SLE itself, but rather the more frequent occurrence of infections, cardiovascular disorders, malignancies or drug-induced complications.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

49,54 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

Table I
Table II

References

  1. Cervera R, Khamashta MA, Hughes GR. The Euro-lupus project: epidemiology of systemic lupus erythematosus in Europe. Lupus 2009 Sep; 18(10): 869–74

    PubMed  Article  CAS  Google Scholar 

  2. Boddaert J, Huong DL, Amoura Z, et al. Late-onset systemic lupus erythematosus: a personal series of 47 patients and pooled analysis of 714 cases in the literature. Medicine (Baltimore) 2004 Nov; 83(6): 348–59

    Article  Google Scholar 

  3. Costallat LT, Coimbra AM. Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset. Clin Exp Rheumatol 1994 Nov–Dec; 12(6): 603–7

    PubMed  CAS  Google Scholar 

  4. Domenech I, Aydintug O, Cervera R, et al. Systemic lupus erythematosus in 50 year olds. Postgrad Med J 1992 Jun; 68(800): 440–4

    PubMed  Article  CAS  Google Scholar 

  5. Font J, Pallares L, Cervera R, et al. Systemic lupus erythematosus in the elderly: clinical and immunological characteristics. Ann Rheum Dis 1991 Oct; 50(10): 702–5

    PubMed  Article  CAS  Google Scholar 

  6. Hashimoto H, Tsuda H, Hirano T, et al. Differences in clinical and immunological findings of systemic lupus erythematosus related to age. J Rheumatol 1987 Jun; 14(3): 497–501

    PubMed  CAS  Google Scholar 

  7. Takayasu V, Bonfa E, Levy Neto M, et al. Systemic lupus erythematosus in the aged: clinical and laboratory characteristics [in Portuguese]. Rev Hosp Clin Fac Med Sao Paulo 1992 Jan–Feb; 47(1): 6–9

    PubMed  CAS  Google Scholar 

  8. Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore) 1993 Mar; 72(2): 113–24

    CAS  Google Scholar 

  9. Dubois EL, Tuffanelli DL. Clinical manifestations of systemic lupus erythematosus: computer analysis of 520 cases. JAMA 1964 Oct 12; 190: 104–11

    PubMed  Article  CAS  Google Scholar 

  10. Harvey AM, Shulman LE, Tumulty PA, et al. Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine (Baltimore) 1954 Dec; 33(4): 291–437

    Article  CAS  Google Scholar 

  11. Antolin J, Amerigo MJ, Cantabrana A, et al. Systemic lupus erythematosus: clinical manifestations and immunological parameters in 194 patients. Subgroup classification of SLE. Clin Rheumatol 1995 Nov; 14(6): 678–85

    PubMed  Article  CAS  Google Scholar 

  12. Baker SB, Rovira JR, Campion EW, et al. Late onset systemic lupus erythematosus. Am J Med 1979 May; 66(5): 727–32

    PubMed  Article  CAS  Google Scholar 

  13. Ballou SP, Khan MA, Kushner I. Clinical features of systemic lupus erythematosus: differences related to race and age of onset. Arthritis Rheum 1982 Jan; 25(1): 55–60

    PubMed  Article  CAS  Google Scholar 

  14. Dimant J, Ginzier EM, Schlesinger M, et al. Systemic lupus erythematosus in the older age group: computer analysis. J Am Geriatr Soc 1979 Feb; 27(2): 58–61

    PubMed  CAS  Google Scholar 

  15. Foad BS, Sheon RP, Kirsner AB. Systemic lupus erythematosus in the elderly. Arch Intern Med 1972 Nov; 130(5): 743–6

    PubMed  Article  CAS  Google Scholar 

  16. Joseph RR, Zarafonetis CJ. Clinical onset of lupus erythematosus in the older age group. J Am Geriatr Soc 1964 Aug; 12: 787–99

    PubMed  CAS  Google Scholar 

  17. Wilson HA, Hamilton ME, Spyker DA, et al. Age influences the clinical and serologic expression of systemic lupus erythematosus. Arthritis Rheum 1981 Oct; 24(10): 1230–5

    PubMed  Article  CAS  Google Scholar 

  18. Bertoli AM, Alarcon GS, Calvo-Alen J, et al. Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical [corrected] features, course, and outcome in patients with late-onset disease. Arthritis Rheum 2006 May; 54(5): 1580–7

    PubMed  Article  Google Scholar 

  19. Catoggio LJ, Skinner RP, Smith G, et al. Systemic lupus erythematosus in the elderly: clinical and serological characteristics. J Rheumatol 1984 Apr; 11(2): 175–81

    PubMed  CAS  Google Scholar 

  20. Maddison P, Farewell V, Isenberg D, et al. The rate and pattern of organ damage in late onset systemic lupus erythematosus. J Rheumatol 2002 May; 29(5): 913–7

    PubMed  Google Scholar 

  21. Maddison PJ. Systemic lupus erythematosus in the elderly. J Rheumatol Suppl 1987 Jun; 14Suppl. 13: 182–7

    PubMed  Google Scholar 

  22. Appenzeller S, Pereira DA, Costallat LT. Greater accrual damage in late-onset systemic lupus erythematosus: a long-term follow-up study. Lupus 2008 Nov; 17(11): 1023–8

    PubMed  Article  CAS  Google Scholar 

  23. Gaujard S, Broussolle C, Cathebras P, et al. Systemic lupus erythematosus with disease onset after age 65 [in French]. Rev Med Interne 2003 May; 24(5): 288–94

    PubMed  Article  CAS  Google Scholar 

  24. Karoubi Nordon E, Hayem G, Mentres F, et al. Late onset systemic lupus erythematosus: a new approach. Lupus 2007; 16(12): 1011–4

    PubMed  Article  CAS  Google Scholar 

  25. Formiga F, Moga I, Pac M, et al. Mild presentation of systemic lupus erythematosus in elderly patients assessed by SLEDAI: SLE Disease Activity Index. Lupus 1999; 8(6): 462–5

    PubMed  Article  CAS  Google Scholar 

  26. Ho CT, Mok CC, Lau CS, et al. Late onset systemic lupus erythematosus in southern Chinese. Ann Rheum Dis 1998 Jul; 57(7): 437–40

    PubMed  Article  CAS  Google Scholar 

  27. Mak SK, Lam EK, Wong AK. Clinical profile of patients with late-onset SLE: not a benign subgroup. Lupus 1998; 7(1): 23–8

    PubMed  Article  CAS  Google Scholar 

  28. Gossat DM, Walls RS. Systemic lupus erythematosus in later life. Med J Aust 1982 Apr 3; 1(7): 297–9

    PubMed  CAS  Google Scholar 

  29. Lalani S, Pope J, de Leon F, et al. Clinical features and prognosis of late-onset systemic lupus erythematosus: results from the 1000 faces of lupus study. J Rheumatol 2009 Jan; 37(1): 38–44

    PubMed  Article  Google Scholar 

  30. Padovan M, Govoni M, Castellino G, et al. Late onset systemic lupus erythematosus: no substantial differences using different cut-off ages. Rheumatol Int 2007 Jun; 27(8): 735–41

    PubMed  Article  Google Scholar 

  31. Shaikh SK, Wang F. Late-onset systemic lupus erythematosus: clinical and immunological characteristics. Med J Malaysia 1995 Mar; 50(1): 25–31

    PubMed  CAS  Google Scholar 

  32. Koh ET, Boey ML. Late onset lupus: a clinical and immunological study in a predominantly Chinese population. J Rheumatol 1994 Aug; 21(8): 1463–7

    PubMed  CAS  Google Scholar 

  33. Pu SJ, Luo SF, Wu YJ, et al. The clinical features and prognosis of lupus with disease onset at age 65 and older. Lupus 2000; 9(2): 96–100

    PubMed  Article  CAS  Google Scholar 

  34. Sayarlioglu M, Cefle A, Kamali S, et al. Characteristics of patients with late onset systemic lupus erythematosus in Turkey. Int J Clin Pract 2005 Feb; 59(2): 183–7

    PubMed  Article  CAS  Google Scholar 

  35. Ward MM, Polisson RP. A meta-analysis of the clinical manifestations of older-onset systemic lupus erythematosus. Arthritis Rheum 1989 Oct; 32(10): 1226–32

    PubMed  Article  CAS  Google Scholar 

  36. Jacobsen S, Petersen J, Ullman S, et al. A multicentre study of 513 Danish patients with systemic lupus erythematosus. II. Disease mortality and clinical factors of prognostic value. Clin Rheumatol 1998; 17(6): 478–84

    PubMed  Article  CAS  Google Scholar 

  37. Jacobsen S, Petersen J, Ullman S, et al. A multicentre study of 513 Danish patients with systemic lupus erythematosus: I. Disease manifestations and analyses of clinical subsets. Clin Rheumatol 1998; 17(6): 468–77

    PubMed  Article  CAS  Google Scholar 

  38. Studenski S, Allen NB, Caldwell DS, et al. Survival in systemic lupus erythematosus: a multivariate analysis of demographic factors. Arthritis Rheum 1987 Dec; 30(12): 1326–32

    PubMed  Article  CAS  Google Scholar 

  39. Maddock Jr RK. Incidence of systemic lupus erythematosus by age and sex. JAMA 1965 Jan 11; 191: 137–8

    PubMed  Article  Google Scholar 

  40. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997 Sep; 40(9): 1725

    PubMed  Article  CAS  Google Scholar 

  41. 1997 update of the 1982 American College of Rheumatology revised criteria for classification of systemic lupus erythematosus. Atlanta (GA): American College of Rheumatology [online]. Available from URL: http://www.rheumatology.org/practice/clinical/classification/SLE/1997_update_of_the_1982_acr_revised_criteria_for_classification_of_sle.pdf [Accessed 2011 Dec 20]

  42. Katz U, Zandman-Goddard G. Drug-induced lupus: an update. Autoimmun Rev 2010 Nov; 10(1): 46–50

    PubMed  Article  CAS  Google Scholar 

  43. Jonsson H, Nived O, Sturfelt G. The effect of age on clinical and serological manifestations in unselected patients with systemic lupus erythematosus. J Rheumatol 1988 Mar; 15(3): 505–9

    PubMed  CAS  Google Scholar 

  44. Janwityanujit S, Totemchokchyakarn K, Verasertniyom O, et al. Age-related differences on clinical and immunological manifestations of SLE. Asian Pac J Allergy Immunol 1995 Dec; 13(2): 145–9

    PubMed  CAS  Google Scholar 

  45. Manoussakis MN, Georgopoulou C, Zintzaras E, et al. Sjogren’s syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjogren’s syndrome. Arthritis Rheum 2004 Mar; 50(3): 882–91

    PubMed  Article  Google Scholar 

  46. Huong DL, Papo T, Beaufils H, et al. Renal involvement in systemic lupus erythematosus: a study of 180 patients from a single center. Medicine (Baltimore) 1999 May; 78(3): 148–66

    Article  CAS  Google Scholar 

  47. Reveille JD, Bartolucci A, Alarcon GS. Prognosis in systemic lupus erythematosus: negative impact of increasing age at onset, black race, and thrombocytopenia, as well as causes of death. Arthritis Rheum 1990 Jan; 33(1): 37–48

    PubMed  Article  CAS  Google Scholar 

  48. Rovensky J, Tuchynova A. Systemic lupus erythematosus in the elderly. Autoimmun Rev 2008 Jan; 7(3): 235–9

    PubMed  Article  CAS  Google Scholar 

  49. Scalzi LV, Hollenbeak CS, Wang L. Racial disparities in age at time of cardiovascular events and cardiovascular-related death in patients with systemic lupus erythematosus. Arthritis Rheum 2010 Sep; 62(9): 2767–75

    PubMed  Article  Google Scholar 

  50. Tan EM, Feltkamp TE, Smolen JS, et al. Range of anti-nuclear antibodies in “healthy” individuals. Arthritis Rheum 1997 Sep; 40(9): 1601–11

    PubMed  Article  CAS  Google Scholar 

  51. Arnaud L, Zahr N, Costedoat-Chalumeau N, et al. The importance of assessing medication exposure to the definition of refractory disease in systemic lupus erythematosus. Autoimmun Rev 2011 Sep; 10(11): 674–8

    PubMed  Article  CAS  Google Scholar 

  52. Costedoat-Chalumeau N, Leroux G, Piette JC, et al. Why all systemic lupus erythematosus patients should be given hydroxychloroquine treatment? Joint Bone Spine 2009 Jan; 77(1): 4–5

    PubMed  Article  Google Scholar 

  53. Costedoat-Chalumeau N, Amoura Z, Hulot JS, et al. Low blood concentration of hydroxychloroquine is a marker for and predictor of disease exacerbations in patients with systemic lupus erythematosus. Arthritis Rheum 2006 Oct; 54(10): 3284–90

    PubMed  Article  CAS  Google Scholar 

  54. Marmor MF, Kellner U, Lai TY, et al. Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology 2011 Feb; 118(2): 415–22

    PubMed  Article  Google Scholar 

  55. Appel GB, Contreras G, Dooley MA, et al. Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis. J Am Soc Nephrol 2009 May; 20(5): 1103–12

    PubMed  Article  CAS  Google Scholar 

  56. Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus 2011 Feb; 20(2): 206–18

    PubMed  Article  CAS  Google Scholar 

  57. Merrill JT, Neuwelt CM, Wallace DJ, et al. Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Arthritis Rheum 2010 Jan; 62(1): 222–33

    PubMed  Article  CAS  Google Scholar 

  58. Furie R, Looney J, Rovin B, et al. Efficacy and safety of rituximab (RTX) in patients (PTS) with proliferative lupus nephritis (LN): results from the randomized, double-blind phase III LUNAR study. Ann Rheum Dis 2010; 69Suppl. 3: 549

    Google Scholar 

  59. Navarra SV, Guzman RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet 2011 Feb 26; 377(9767): 721–31

    PubMed  Article  CAS  Google Scholar 

  60. Furie RA, Petri MA, Wallace DJ, et al. Novel evidence-based systemic lupus erythematosus responder index. Arthritis Rheum 2009 Sep 15; 61(9): 1143–51

    PubMed  Article  CAS  Google Scholar 

Download references

Acknowledgements

No sources of funding were used to assist in the preparation of this article. The authors have no conflicts of interest that are directly relevant to the content of this article.

Author information

Affiliations

  1. Department of Internal Medicine, French Reference Center for Systemic Lupus Erythematosus, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

    Laurent Arnaud, Alexis Mathian, Jacques Boddaert &  Zahir Amoura MD

  2. Institut National de la Santé et de la Recherche Médicale, INSERM UMR-S 945, Paris, France

    Laurent Arnaud, Alexis Mathian &  Zahir Amoura MD

  3. Université Pierre et Marie Curie, UPMC Univ Paris 06, Paris, France

    Alexis Mathian, Jacques Boddaert &  Zahir Amoura MD

  4. Department of Geriatric Medicine, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

    Jacques Boddaert

Authors
  1. Laurent Arnaud
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Alexis Mathian
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jacques Boddaert
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Zahir Amoura MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Zahir Amoura MD.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Arnaud, L., Mathian, A., Boddaert, J. et al. Late-Onset Systemic Lupus Erythematosus. Drugs Aging 29, 181–189 (2012). https://doi.org/10.2165/11598550-000000000-00000

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.2165/11598550-000000000-00000

Keywords

  • Systemic Lupus Erythematosus
  • Systemic Lupus Erythematosus Patient
  • Lupus Nephritis
  • Belimumab
  • Polymyalgia Rheumatica