Connect
MJA
MJA

Systemic lupus erythematosus: an update

Vera Golder and Alberta Hoi
Med J Aust 2017; 206 (5): . || doi: 10.5694/mja16.01229
Published online: 20 March 2017

Summary

  • Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease predominantly affecting women of childbearing age.
  • New classification criteria for SLE have greater sensitivity and therefore improve the diagnostic certainty for some patients, especially those who may previously have been labelled as having undifferentiated symptoms.
  • Uncontrolled disease activity leads to irreversible end-organ damage, which in turn increases the risk of premature death; early and sustained control of disease activity can usually be achieved by conventional immunosuppressant therapy.
  • The development of biological therapy lags behind that for other rheumatic diseases, with belimumab being the only targeted therapy approved by the Therapeutic Goods Administration.
  • “Treat-to-target” concepts are changing trial design and clinical practice, with evidence-based definition of response criteria in the form of remission and low disease activity now on the horizon.
  • While new therapies are awaited, research should also focus on optimising the use of current therapy and improving the quality of care of patients with SLE.


  • Monash University, Melbourne, VIC


Correspondence: alberta.hoi@monash.edu

Acknowledgements: 

Vera Golder holds a postgraduate scholarship from the National Health and Medical Research Council (NHMRC, APP1093545).

Competing interests:

Alberta Hoi has received funding from GlaxoSmithKline, MOVE Muscle, Bone and Joint Health, Astra-Zeneca and UCB as unrestricted educational grants for the development of the Australian Lupus Registry and Biobank.

  • 1. Urowitz MB, Gladman DD, Ibanez D, et al. Evolution of disease burden over five years in a multicenter inception systemic lupus erythematosus cohort. Arthritis Care Res (Hoboken) 2012; 64: 132-137.
  • 2. Nikpour M, Urowitz MB, Ibanez D, Gladman DD. Frequency and determinants of flare and persistently active disease in systemic lupus erythematosus. Arthritis Rheum 2009; 61: 1152-1158.
  • 3. Petri M, Purvey S, Fang H, Magder LS. Predictors of organ damage in systemic lupus erythematosus: the Hopkins Lupus Cohort. Arthritis Rheum 2012; 64: 4021-4028.
  • 4. Alarcon GS, McGwin G Jr, Bastian HM, et al. Systemic lupus erythematosus in three ethnic groups. VII [correction of VIII]. Predictors of early mortality in the LUMINA cohort. LUMINA Study Group. Arthritis Rheum 2001; 45: 191-202.
  • 5. Mok CC, Ho LY, Cheung MY, et al. Effect of disease activity and damage on quality of life in patients with systemic lupus erythematosus: a 2-year prospective study. Scand J Rheumatol 2009; 38: 121-127.
  • 6. Jolly M. How does quality of life of patients with systemic lupus erythematosus compare with that of other common chronic illnesses? J Rheumatol 2005; 32: 1706-1708.
  • 7. Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012; 64: 2677-2686.
  • 8. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725.
  • 9. Bortoluzzi A, Furini F, Campanaro F, Govoni M. Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up. Lupus 2016; doi:10.1177/0961203316671814 [Epub ahead of print].
  • 10. Tan EM, Feltkamp TE, Smolen JS, et al. Range of antinuclear antibodies in “healthy” individuals. Arthritis Rheum 1997; 40: 1601-1611.
  • 11. Alba P, Bento L, Cuadrado MJ, et al. Anti-dsDNA, anti-Sm antibodies, and the lupus anticoagulant: significant factors associated with lupus nephritis. Ann Rheum Dis 2003; 62: 556-560.
  • 12. Derksen RH, Bast EJ, Strooisma T, Jacobs JW. A comparison between the Farr radioimmunoassay and a new automated fluorescence immunoassay for the detection of antibodies against double stranded DNA in serum. Ann Rheum Dis 2002; 61: 1099-1102.
  • 13. González-Naranjo LA, Betancur OM, Alarcón GS, et al. Features associated with hematologic abnormalities and their impact in patients with systemic lupus erythematosus: Data from a multiethnic Latin American cohort. Semin Arthritis Rheum 2016; 45: 675-683.
  • 14. Golder V, Connelly K, Staples M, et al. Association of Asian ethnicity with disease activity in SLE: an observational study from the Monash Lupus Clinic. Lupus 2013; 22: 1425-1430.
  • 15. Vincent FB, Bourke P, Morand EF, et al. Focus on systemic lupus erythematosus in indigenous Australians: towards a better understanding of autoimmune diseases. Intern Med J 2013; 43: 227-234.
  • 16. Ugarte-Gil MF, Alarcon GS. Incomplete systemic lupus erythematosus: early diagnosis or overdiagnosis? Arthritis Care Res (Hoboken) 2016; 68: 285-287.
  • 17. Condon MB, Ashby D, Pepper RJ, et al. Prospective observational single-centre cohort study to evaluate the effectiveness of treating lupus nephritis with rituximab and mycophenolate mofetil but no oral steroids. Ann Rheum Dis 2013; 72: 1280-1286.
  • 18. van Staa TP, Leufkens HG, Cooper C. The epidemiology of corticosteroid-induced osteoporosis: a meta-analysis. Osteoporos Int 2002; 13: 777-787.
  • 19. Gurwitz JH, Bohn RL, Glynn RJ, et al. Glucocorticoids and the risk for initiation of hypoglycemic therapy. Arch Intern Med 1994; 154: 97-101.
  • 20. Ruiz-Irastorza G, Olivares N, Ruiz-Arruza I, et al. Predictors of major infections in systemic lupus erythematosus. Arthritis Res Ther 2009; 11: R109.
  • 21. Ruiz-Arruza I, Ugarte A, Cabezas-Rodriguez I, et al. Glucocorticoids and irreversible damage in patients with systemic lupus erythematosus. Rheumatology (Oxford) 2014; 53: 1470-1476.
  • 22. Thamer M, Hernan MA, Zhang Y, et al. Prednisone, lupus activity, and permanent organ damage. J Rheumatol 2009; 36: 560-564.
  • 23. Alarcón GS, McGwin G, Bertoli AM, et al. Effect of hydroxychloroquine on the survival of patients with systemic lupus erythematosus: data from LUMINA, a multiethnic US cohort (LUMINA L). Ann Rheum Dis 2007; 66: 1168-1172.
  • 24. Kasitanon N, Fine DM, Haas M, et al. Hydroxychloroquine use predicts complete renal remission within 12 months among patients treated with mycophenolate mofetil therapy for membranous lupus nephritis. Lupus 2006; 15: 366-370.
  • 25. Marmor MF, Kellner U, Lai TY, et al. Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 Revision). Ophthalmology 2016; 123: 1386-1394.
  • 26. Ding HJ, Denniston AK, Rao VK, Gordon C. Hydroxychloroquine-related retinal toxicity. Rheumatology (Oxford) 2016; 55: 957-967.
  • 27. Liu LL, Jiang Y, Wang LN, et al. Efficacy and safety of mycophenolate mofetil versus cyclophosphamide for induction therapy of lupus nephritis: a meta-analysis of randomized controlled trials. Drugs 2012; 72: 1521-1533.
  • 28. Tselios K, Gladman DD, Su J, Urowitz MB. Mycophenolate mofetil in nonrenal manifestations of systemic lupus erythematosus: an observational cohort study. J Rheumatol 2016; 43: 552-558.
  • 29. Houssiau FA, Vasconcelos C, D’Cruz D, et al. The 10-year follow-up data of the Euro-Lupus Nephritis Trial comparing low-dose and high-dose intravenous cyclophosphamide. Ann Rheum Dis 2010; 69: 61-64.
  • 30. Franklyn K, Hoi A, Nikpour M, Morand EF. The need to define treatment goals for systemic lupus erythematosus. Nat Rev Rheumatol 2014; 10: 567-571.
  • 31. Hoi A, Littlejohn G. Is there still a role for abatacept in the treatment of lupus? Expert Opin Biol Ther 2014; 14: 1345-1350.
  • 32. Leone A, Sciascia S, Kamal A, Khamashta M. Biologicals for the treatment of systemic lupus erythematosus: current status and emerging therapies. Expert Rev Clin Immunol 2015; 11: 109-116.
  • 33. 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; 62: 222-233.
  • 34. 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; 377: 721-731.
  • 35. van Vollenhoven RF, Petri MA, Cervera R, et al. Belimumab in the treatment of systemic lupus erythematosus: high disease activity predictors of response. Ann Rheum Dis 2012; 71: 1343-1349.
  • 36. Furie R, Merrill J, Werth VP, et al. Anifrolumab, an anti-interferon alpha receptor monoclonal antibody, in moderate to severe systemic lupus erythematosus (SLE) [abstract]. Arthritis Rheumatol 2015; 67 Suppl 10. http://acrabstracts.org/abstract/anifrolumab-an-anti-interferon-alpha-receptor-monoclonal-antibody-in-moderate-to-severe-systemic-lupus-erythematosus-sle/ (accessed Jan 2017).
  • 37. Connelly KL, Kandane-Rathnayake R, Hoi A, et al. Association of MIF, but not type I interferon-induced chemokines, with increased disease activity in Asian patients with systemic lupus erythematosus. Sci Rep 2016; 6: 29909. doi: 10.1038/srep29909.
  • 38. Kennedy WP, Maciuca R, Wolslegel K, et al. Association of the interferon signature metric with serological disease manifestations but not global activity scores in multiple cohorts of patients with SLE. Lupus Sci Med 2015; 2: e000080.
  • 39. van Vollenhoven RF, Mosca M, Bertsias G, et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis 2014; 73: 958-967.
  • 40. Franklyn K, Lau CS, Navarra SV, et al. Definition and initial validation of a Lupus Low Disease Activity State (LLDAS). Ann Rheum Dis 2016; 75: 1615-1621.
  • 41. Golder V, Kandane-Rathnayake R, Hoi AY, et al. Frequency and predictors of the lupus low disease activity state in a multi-national and multi-ethnic cohort. Arthritis Res Ther 2016; 18: 260.
  • 42. Gladman DD, Ibanez D, Ruiz I, Urowitz MB. Recommendations for frequency of visits to monitor systemic lupus erythematosus in asymptomatic patients: data from an observational cohort study. J Rheumatol 2013; 40: 630-633.
  • 43. Mosca M, Tani C, Aringer M, et al. European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies. Ann Rheum Dis 2010; 69: 1269-1274.
  • 44. Yazdany J, Panopalis P, Gillis JZ, et al. A quality indicator set for systemic lupus erythematosus. Arthritis Rheum 2009; 61: 370-377.
  • 45. Yazdany J, Trupin L, Schmajuk G, et al. Quality of care in systemic lupus erythematosus: the association between process and outcome measures in the Lupus Outcomes Study. BMJ Qual Saf 2014; 23: 659-666.
  • 46. Ward MM. Hospital experience and mortality in patients with systemic lupus erythematosus: which patients benefit most from treatment at highly experienced hospitals? J Rheumatol 2002; 29: 1198-1206.
  • 47. Bartels CM, Buhr KA, Goldberg JW, et al. Mortality and cardiovascular burden of systemic lupus erythematosus in a US population-based cohort. J Rheumatol 2014; 41: 680-687.
  • 48. Magder LS, Petri M. Incidence of and risk factors for adverse cardiovascular events among patients with systemic lupus erythematosus. Am J Epidemiol 2012; 176: 708-719.
  • 49. Urowitz MB, Ibanez D, Su J, Gladman DD. Modified Framingham Risk Factor Score for systemic lupus erythematosus. J Rheumatol 2016; 43: 875-879.
  • 50. Wang X, Yan S, Liu C, et al. Fracture risk and bone mineral density levels in patients with systemic lupus erythematosus: a systematic review and meta-analysis. Osteoporos Int 2016; 27: 1413-1423.

Author

remove_circle_outline Delete Author
add_circle_outline Add Author

Comment
Do you have any competing interests to declare? *

I/we agree to assign copyright to the Medical Journal of Australia and agree to the Conditions of publication *
I/we agree to the Terms of use of the Medical Journal of Australia *
Email me when people comment on this article

Online responses are no longer available. Please refer to our instructions for authors page for more information.