Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

Prevalence and burden of pediatric-onset systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease with a highly variable clinical course. Pediatric-onset SLE (pSLE) represents 10–20% of all SLE cases, and is associated with higher disease severity, including more-rapid damage accrual, than adult-onset SLE. As in adults, pSLE disease expression varies according to ethnicity, with a milder disease course in white patients. The majority of pSLE patients will have developed damage within 5–10 years of disease onset, most frequently involving the musculoskeletal, ocular, renal and neuropsychiatric systems. Owing to improvements in disease management and recognition over the past 20–30 years, patients now live longer, but as a result have increased disease damage. Premature atherosclerosis and osteoporosis have become increasingly prevalent morbidities in pSLE patients. Early atherosclerosis leads to a considerable rise in cardiovascular and cerebrovascular events, and failure to develop adequate peak bone mass during adolescence—a crucial period of bone accrual—is likely to lead to early osteoporosis and fractures. Patients with pSLE have an incurable, potentially devastating disease that occurs during a vulnerable period of psychosocial development, leading to specific and unique psychosocial stressors. Additional large, long-term follow-up studies in pSLE are needed to better understand the disease prognosis and to facilitate development of tailored treatments.

Key Points

  • Pediatric-onset systemic lupus erythematosus (pSLE) follows a more severe clinical course than adult-onset SLE, with higher disease activity and more-rapid damage accrual

  • Despite improved disease management, a considerable proportion of pSLE patients still die before the age of 30 years

  • Premature atherosclerosis and bone-related complications, including osteoporosis and osteonecrosis, have become important morbidities in SLE

  • Optimal control of disease activity is necessary to minimize the risk for damage secondary to disease and therapy-related complications and to decrease mortality

  • Disease expression and outcome differs between white and non-white patients with pSLE, but larger studies are needed to verify the effect of ethnicity on disease course and outcome

  • Transfer of adolescents with pSLE to a dedicated rheumatologist with specific knowledge of the characteristics of pSLE and the unique needs of these patients is essential to achieving good long-term outcomes

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

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Ramírez Gómez, L. A. et al. Childhood systemic lupus erythematosus in Latin America. The GLADEL experience in 230 children. Lupus 17, 596–604 (2008).

    Article  PubMed  Google Scholar 

  2. Carreño, L. et al. Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus 8, 287–292 (1999).

    Article  PubMed  Google Scholar 

  3. Font, J. et al. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann. Rheum. Dis. 57, 456–459 (1998).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Mok, C. C., Mak, A., Chu, W. P., To, C. H. & Wong, S. N. Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups. Medicine (Baltimore) 84, 218–224 (2005).

    Article  Google Scholar 

  5. Lee, B. W. et al. A 10 year review of systemic lupus erythematosus in Singapore children. Aust. Paediatr. J. 23, 163–165 (1987).

    CAS  PubMed  Google Scholar 

  6. Brunner, H. I., Silverman, E. D., To, T., Bombardier, C. & Feldman, B. M. Risk factors for damage in childhood-onset systemic lupus erythematosus: cumulative disease activity and medication use predict disease damage. Arthritis Rheum. 46, 436–444 (2002).

    Article  PubMed  Google Scholar 

  7. Tucker, L. B., Menon, S., Schaller, J. G. & Isenberg, D. A. Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br. J. Rheumatol. 34, 866–872 (1995).

    Article  CAS  PubMed  Google Scholar 

  8. Hiraki, L. T. et al. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J. Pediatr. 152, 550–556 (2008).

    Article  PubMed  Google Scholar 

  9. Pluchinotta, F. R. et al. Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus 16, 550–555 (2007).

    Article  CAS  PubMed  Google Scholar 

  10. Wong, S. N. et al. Lupus nephritis in Chinese children—a territory-wide cohort study in Hong Kong. Pediatr. Nephrol. 21, 1104–1112 (2006).

    Article  PubMed  Google Scholar 

  11. McCarty, D. J. et al. Incidence of systemic lupus erythematosus. Race and gender differences. Arthritis Rheum. 38, 1260–1270 (1995).

    Article  CAS  PubMed  Google Scholar 

  12. Peschken, C. A. et al. The 1,000 Canadian faces of lupus: determinants of disease outcome in a large multiethnic cohort. J. Rheumatol. 36, 1200–1208 (2009).

    Article  PubMed  Google Scholar 

  13. Hiraki, L. T. et al. Ethnic differences in pediatric systemic lupus erythematosus. J. Rheumatol. 3 6, 2539–2546 (2009).

    Article  Google Scholar 

  14. Cooper, G. S. et al. Differences by race, sex and age in the clinical and immunologic features of recently diagnosed systemic lupus erythematosus patients in the southeastern United States. Lupus 11, 161–167 (2002).

    Article  CAS  PubMed  Google Scholar 

  15. Huang, J. L., Yao, T. C. & See, L. C. Prevalence of pediatric systemic lupus erythematosus and juvenile chronic arthritis in a Chinese population: a nation-wide prospective population-based study in Taiwan. Clin. Exp. Rheumatol. 22, 776–780 (2004).

    CAS  PubMed  Google Scholar 

  16. See, Y., Koh, E. T. & Boey, M. L. One hundred and seventy cases of childhood-onset rheumatological disease in Singapore. Ann. Acad. Med. Singapore 27, 496–502 (1998).

    CAS  PubMed  Google Scholar 

  17. Yang, L. Y., Chen, W. P. & Lin, C. Y. Lupus nephritis in children—a review of 167 patients. Pediatrics 94, 335–340 (1994).

    CAS  PubMed  Google Scholar 

  18. Tucker, L. B. et al. Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case–control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus 17, 314–322 (2008).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Hersh, A. O. et al. Differences in long-term disease activity and treatment of adult patients with childhood- and adult-onset systemic lupus erythematosus. Arthritis Rheum. 61, 13–20 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  20. Pons-Estel, G. J., Alarcón, G. S., Scofield, L., Reinlib, L. & Cooper, G. S. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin. Arthritis Rheum. 39, 257–268 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  21. Huemer, C. et al. Incidence of pediatric rheumatic diseases in a regional population in Austria. J. Rheumatol. 28, 2116–2119 (2001).

    CAS  PubMed  Google Scholar 

  22. Denardo, B. A., Tucker, L. B., Miller, L. C., Szer, I. S. & Schaller, J. G. Demography of a regional pediatric rheumatology patient population. Affiliated Children's Arthritis Centers of New England. J. Rheumatol. 21, 1553–1561 (1994).

    CAS  PubMed  Google Scholar 

  23. Kaipiainen-Seppänen, O. & Savolainen, A. Incidence of chronic juvenile rheumatic diseases in Finland during 1980–1990. Clin. Exp. Rheumatol. 14, 441–444 (1996).

    PubMed  Google Scholar 

  24. Houghton, K. M., Page, J., Cabral, D. A., Petty, R. E. & Tucker, L. B. Systemic lupus erythematosus in the pediatric North American Native population of British Columbia. J. Rheumatol. 33, 161–163 (2006).

    PubMed  Google Scholar 

  25. Malleson, P. N., Fung, M. Y. & Rosenberg, A. M. The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry. J. Rheumatol. 23, 1981–1987 (1996).

    CAS  PubMed  Google Scholar 

  26. Bahíllo, M. P. et al. Incidence and prevalence of type 1 diabetes in children aged <15 yr in Castilla-Leon (Spain). Pediatr. Diabetes 8, 369–373 (2007).

    Article  PubMed  Google Scholar 

  27. Janwityanujit, S., Totemchokchyakarn, K., Verasertniyom, O., Vanichapuntu, M. & Vatanasuk, M. Age-related differences on clinical and immunological manifestations of SLE. Asian Pac. J. Allergy Immunol. 13, 145–149 (1995).

    CAS  PubMed  Google Scholar 

  28. Brunner, H. I., Gladman, D. D., Ibañez, D., Urowitz, M. D. & Silverman, E. D. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum. 58, 556–562 (2008).

    Article  PubMed  Google Scholar 

  29. Hoffman, I. E. et al. Juvenile-onset systemic lupus erythematosus: different clinical and serological pattern than adult-onset systemic lupus erythematosus. Ann. Rheum. Dis. 68, 412–415 (2009).

    Article  CAS  PubMed  Google Scholar 

  30. Gutiérrez-Suárez, R. et al. A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus. Arthritis Rheum. 54, 2989–2996 (2006).

    Article  PubMed  Google Scholar 

  31. Stoll, T., Stucki, G., Malik, J., Pyke, S. & Isenberg, D. A. Association of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index with measures of disease activity and health status in patients with systemic lupus erythematosus. J. Rheumatol. 24, 309–313 (1997).

    CAS  PubMed  Google Scholar 

  32. Nossent, J. C. SLICC/ACR Damage Index in Afro-Caribbean patients with systemic lupus erythematosus: changes in and relationship to disease activity, corticosteroid therapy, and prognosis. J. Rheumatol. 25, 654–659 (1998).

    CAS  PubMed  Google Scholar 

  33. Alarcón, G. S. et al. Systemic lupus erythematosus in three ethnic groups. IX. Differences in damage accrual. Arthritis Rheum. 44, 2797–2806 (2001).

    Article  PubMed  Google Scholar 

  34. Swaak, A. J. et al. Systemic lupus erythematosus: clinical features in patients with a disease duration of over 10 years, first evaluation. Rheumatology (Oxford) 38, 953–958 (1999).

    Article  CAS  Google Scholar 

  35. Urowitz, M. B., Feletar, M., Bruce, I. N., Ibañez, D. & Gladman, D. D. Prolonged remission in systemic lupus erythematosus. J. Rheumatol. 32, 1467–1472 (2005).

    PubMed  Google Scholar 

  36. Candell Chalom, E. et al. Educational, vocational and socioeconomic status and quality of life in adults with childhood-onset systemic lupus erythematosus. Pediatr. Rheumatol. Online J. 2, 207–226 (2004).

    Google Scholar 

  37. Ravelli, A. et al. Assessment of damage in juvenile-onset systemic lupus erythematosus: a multicenter cohort study. Arthritis Rheum. 49, 501–507 (2003).

    Article  PubMed  Google Scholar 

  38. Bandeira, M. et al. Relationship between damage accrual, disease flares and cumulative drug therapies in juvenile-onset systemic lupus erythematosus. Lupus 15, 515–520 (2006).

    Article  CAS  PubMed  Google Scholar 

  39. Miettunen, P. M. et al. Gender and ethnic origin have no effect on longterm outcome of childhood-onset systemic lupus erythematosus. J. Rheumatol. 31, 1650–1654 (2004).

    PubMed  Google Scholar 

  40. Schanberg, L. E. et al. Premature atherosclerosis in pediatric systemic lupus erythematosus: risk factors for increased carotid intima-media thickness in the atherosclerosis prevention in pediatric lupus erythematosus cohort. Arthritis Rheum. 60, 1496–1507 (2009).

    Article  PubMed  PubMed Central  Google Scholar 

  41. Gladman, D. et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum. 39, 363–369 (1996).

    Article  CAS  PubMed  Google Scholar 

  42. Descloux, E. et al. Paediatric systemic lupus erythematosus: prognostic impact of antiphospholipid antibodies. Rheumatology (Oxford) 47, 183–187 (2008).

    Article  CAS  Google Scholar 

  43. Lilleby, V., Flatø, B. & Førre, O. Disease duration, hypertension and medication requirements are associated with organ damage in childhood-onset systemic lupus erythematosus. Clin. Exp. Rheumatol. 23, 261–269 (2005).

    CAS  PubMed  Google Scholar 

  44. Zonana-Nacach, A. et al. Measurement of damage in 210 Mexican patients with systemic lupus erythematosus: relationship with disease duration. Lupus 7, 119–123 (1998).

    Article  CAS  PubMed  Google Scholar 

  45. Sutcliffe, N., Stoll, T., Pyke, S. & Isenberg, D. A. Functional disability and end organ damage in patients with systemic lupus erythematosus (SLE), SLE and Sjögren's syndrome (SS), and primary SS. J. Rheumatol. 25, 63–68 (1998).

    CAS  PubMed  Google Scholar 

  46. Celermajer, D. S., Thorner, P. S., Baumal, R. & Arbus, G. S. Sex differences in childhood lupus nephritis. Am. J. Dis. Child. 138, 586–588 (1984).

    CAS  PubMed  Google Scholar 

  47. Emre, S. et al. Lupus nephritis in children: prognostic significance of clinicopathological findings. Nephron 87, 118–126 (2001).

    Article  CAS  PubMed  Google Scholar 

  48. Lee, B. S. et al. Clinical outcomes of childhood lupus nephritis: a single center's experience. Pediatr. Nephrol. 22, 222–231 (2007).

    Article  PubMed  Google Scholar 

  49. Hagelberg, S. et al. Longterm followup of childhood lupus nephritis. J. Rheumatol. 29, 2635–2642 (2002).

    PubMed  Google Scholar 

  50. Pattaragarn, A. et al. Systemic lupus erythematosus in Thai children: clinicopathologic findings and outcome in 82 patients. J. Med. Assoc. Thai. 88 (Suppl. 8), S232–S241 (2005).

    PubMed  Google Scholar 

  51. Al-Mayouf, S. M. & Al Sonbul, A. Influence of gender and age of onset on the outcome in children with systemic lupus erythematosus. Clin. Rheumatol. 27, 1159–1162 (2008).

    Article  PubMed  Google Scholar 

  52. Bogdanovic, R. et al. Lupus nephritis in childhood: a review of 53 patients followed at a single center. Pediatr. Nephrol. 19, 36–44 (2004).

    Article  PubMed  Google Scholar 

  53. Baqi, N. et al. Lupus nephritis in children: a longitudinal study of prognostic factors and therapy. J. Am. Soc. Nephrol. 7, 924–929 (1996).

    CAS  PubMed  Google Scholar 

  54. Lau, K. K., Jones, D. P., Hastings, M. C., Gaber, L. W. & Ault, B. H. Short-term outcomes of severe lupus nephritis in a cohort of predominantly African-American children. Pediatr. Nephrol. 21, 655–662 (2006).

    Article  PubMed  Google Scholar 

  55. Singh, S., Devidayal, Minz, R., Nada, R. & Joshi, K. Childhood lupus nephritis: 12 years experience from North India. Rheumatol. Int. 26, 604–607 (2006).

    Article  PubMed  Google Scholar 

  56. Vachvanichsanong, P., Dissaneewate, P. & McNeil, E. Diffuse proliferative glomerulonephritis does not determine the worst outcome in childhood-onset lupus nephritis: a 23-year experience in a single centre. Nephrol. Dial. Transplant. 24, 2729–2734 (2009).

    Article  PubMed  Google Scholar 

  57. Hiraki, L. T., Hamilton, J. & Silverman, E. D. Measuring permanent damage in pediatric systemic lupus erythematosus. Lupus 16, 657–662 (2007).

    Article  CAS  PubMed  Google Scholar 

  58. Björnådal, 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. 31, 713–719 (2004).

    PubMed  Google Scholar 

  59. Ward, M. M. Premature morbidity from cardiovascular and cerebrovascular diseases in women with systemic lupus erythematosus. Arthritis Rheum. 42, 338–346 (1999).

    Article  CAS  PubMed  Google Scholar 

  60. Asanuma, Y. et al. Premature coronary-artery atherosclerosis in systemic lupus erythematosus. N. Engl. J. Med. 349, 2407–2415 (2003).

    Article  CAS  PubMed  Google Scholar 

  61. Manzi, S. 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. 145, 408–415 (1997).

    Article  CAS  PubMed  Google Scholar 

  62. Pons-Estel, G. J. et al. Predictors of cardiovascular damage in patients with systemic lupus erythematosus: data from LUMINA (LXVIII), a multiethnic US cohort. Rheumatology (Oxford) 48, 817–822 (2009).

    Article  Google Scholar 

  63. Roman, M. J. et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N. Engl. J. Med. 349, 2399–2406 (2003).

    Article  CAS  PubMed  Google Scholar 

  64. Petri, M. Detection of coronary artery disease and the role of traditional risk factors in the Hopkins Lupus Cohort. Lupus 9, 170–175 (2000).

    Article  CAS  PubMed  Google Scholar 

  65. Roman, M. J. et al. Rate and determinants of progression of atherosclerosis in systemic lupus erythematosus. Arthritis Rheum. 56, 3412–3419 (2007).

    Article  CAS  PubMed  Google Scholar 

  66. Toloza, S. M. et al. Systemic lupus erythematosus in a multiethnic US cohort (LUMINA). XXIII. Baseline predictors of vascular events. Arthritis Rheum. 50, 3947–3957 (2004).

    Article  PubMed  Google Scholar 

  67. Hahn, B. H. Systemic lupus erythematosus and accelerated atherosclerosis. N. Engl. J. Med. 349, 2379–2380 (2003).

    Article  CAS  PubMed  Google Scholar 

  68. Sandborg, C., Ardoin, S. P. & Schanberg, L. Therapy Insight: cardiovascular disease in pediatric systemic lupus erythematosus. Nat. Clin. Pract. Rheumatol. 4, 258–265 (2008).

    Article  CAS  PubMed  Google Scholar 

  69. Esdaile, J. M. et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum. 44, 2331–2337 (2001).

    Article  CAS  PubMed  Google Scholar 

  70. Ahmad, Y. et al. Subclinical atherosclerosis in systemic lupus erythematosus (SLE): the relative contribution of classic risk factors and the lupus phenotype. Rheumatology (Oxford) 46, 983–988 (2007).

    Article  CAS  Google Scholar 

  71. Tyrrell, P. N., Beyene, J., Benseler, S. M., Sarkissian, T. & Silverman, E. D. Predictors of lipid abnormalities in children with new-onset systemic lupus erythematosus. J. Rheumatol. 34, 2112–2119 (2007).

    CAS  PubMed  Google Scholar 

  72. Doria, A. et al. Risk factors for subclinical atherosclerosis in a prospective cohort of patients with systemic lupus erythematosus. Ann. Rheum. Dis. 62, 1071–1077 (2003).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Petri, M., Lakatta, C., Magder, L. & Goldman, D. Effect of prednisone and hydroxychloroquine on coronary artery disease risk factors in systemic lupus erythematosus: a longitudinal data analysis. Am. J. Med. 96, 254–259 (1994).

    Article  CAS  PubMed  Google Scholar 

  74. Sarkissian, T., Beyenne, J., Feldman, B., Adeli, K. & Silverman, E. The complex nature of the interaction between disease activity and therapy on the lipid profile in patients with pediatric systemic lupus erythematosus. Arthritis Rheum. 54, 1283–1290 (2006).

    Article  CAS  PubMed  Google Scholar 

  75. Lilleby, V. Bone status in juvenile systemic lupus erythematosus. Lupus 16, 580–586 (2007).

    Article  CAS  PubMed  Google Scholar 

  76. Sambrook, P. & Cooper, C. Osteoporosis. Lancet 367, 2010–2018 (2006).

    Article  CAS  PubMed  Google Scholar 

  77. Bailey, D. A., McKay, H. A., Mirwald, R. L., Crocker, P. R. & Faulkner, R. A. A six-year longitudinal study of the relationship of physical activity to bone mineral accrual in growing children: the university of Saskatchewan bone mineral accrual study. J. Bone Miner. Res. 14, 1672–1679 (1999).

    Article  CAS  PubMed  Google Scholar 

  78. [No authors listed] Osteoporosis prevention, diagnosis, and therapy. NIH Consens. Statement 17, 1–45 (2000).

  79. Trapani, S., Civinini, R., Ermini, M., Paci, E. & Falcini, F. Osteoporosis in juvenile systemic lupus erythematosus: a longitudinal study on the effect of steroids on bone mineral density. Rheumatol. Int. 18, 45–49 (1998).

    Article  CAS  PubMed  Google Scholar 

  80. Lilleby, V. et al. Frequency of osteopenia in children and young adults with childhood-onset systemic lupus erythematosus. Arthritis Rheum. 52, 2051–2059 (2005).

    Article  PubMed  Google Scholar 

  81. Lane, N. E. Therapy Insight: osteoporosis and osteonecrosis in systemic lupus erythematosus. Nat. Clin. Pract. Rheumatol. 2, 562–569 (2006).

    Article  CAS  PubMed  Google Scholar 

  82. Alsufyani, K. A. et al. Bone mineral density in children and adolescents with systemic lupus erythematosus, juvenile dermatomyositis, and systemic vasculitis: relationship to disease duration, cumulative corticosteroid dose, calcium intake, and exercise. J. Rheumatol. 32, 729–733 (2005).

    CAS  PubMed  Google Scholar 

  83. Compeyrot-Lacassagne, S. et al. Prevalence and etiology of low bone mineral density in juvenile systemic lupus erythematosus. Arthritis Rheum. 56, 1966–1973 (2007).

    Article  PubMed  Google Scholar 

  84. Regio, P., Bonfá, E., Takayama, L. & Pereira, R. The influence of lean mass in trabecular and cortical bone in juvenile onset systemic lupus erythematosus. Lupus 17, 787–792 (2008).

    Article  PubMed  Google Scholar 

  85. Baim, S. et al. Official Positions of the International Society for Clinical Densitometry and executive summary of the 2007 ISCD Position Development Conference. J. Clin. Densitom. 11, 75–91 (2008).

    Article  PubMed  Google Scholar 

  86. Castro, T. C. et al. Bone mineral density in juvenile systemic lupus erythematosus. Braz. J. Med. Biol. Res. 35, 1159–1163 (2002).

    Article  CAS  PubMed  Google Scholar 

  87. Wang, M. C. et al. Diet in midpuberty and sedentary activity in prepuberty predict peak bone mass. Am. J. Clin. Nutr. 77, 495–503 (2003).

    Article  CAS  PubMed  Google Scholar 

  88. Greer, F. R. & Krebs, N. F. Optimizing bone health and calcium intakes of infants, children, and adolescents. Pediatrics 117, 578–585 (2006).

    Article  PubMed  Google Scholar 

  89. Specker, B. & Binkley, T. Randomized trial of physical activity and calcium supplementation on bone mineral content in 3- to 5-year-old children. J. Bone Miner. Res. 18, 885–892 (2003).

    Article  CAS  PubMed  Google Scholar 

  90. Bachrach, L. K. & Ward, L. M. Clinical review 1: Bisphosphonate use in childhood osteoporosis. J. Clin. Endocrinol. Metab. 94, 400–409 (2009).

    Article  CAS  PubMed  Google Scholar 

  91. Mok, C. C., Lau, C. S. & Wong, R. W. Risk factors for avascular bone necrosis in systemic lupus erythematosus. Br. J. Rheumatol. 37, 895–900 (1998).

    Article  CAS  PubMed  Google Scholar 

  92. Assouline-Dayan, Y., Chang, C., Greenspan, A., Shoenfeld, Y. & Gershwin, M. E. Pathogenesis and natural history of osteonecrosis. Semin. Arthritis Rheum. 32, 94–124 (2002).

    Article  PubMed  Google Scholar 

  93. Ibañez, D., Gladman, D. D. & Urowitz, M. B. Adjusted mean Systemic Lupus Erythematosus Disease Activity Index-2K is a predictor of outcome in SLE. J. Rheumatol. 32, 824–827 (2005).

    PubMed  Google Scholar 

  94. Campos, L. M., Kiss, M. H., D'Amico, E. A. & Silva, C. A. Antiphospholipid antibodies and antiphospholipid syndrome in 57 children and adolescents with systemic lupus erythematosus. Lupus 12, 820–826 (2003).

    Article  CAS  PubMed  Google Scholar 

  95. Castro, F. P. Jr, Hartz, R. S., Frigon, V. & Whitecloud, T. S. 3rd. Aortic thrombosis after lumbar spine surgery. J. Spinal Disord. 13, 538–540 (2000).

    Article  PubMed  Google Scholar 

  96. Garberina, M. J., Berend, K. R., Gunneson, E. E. & Urbaniak, J. R. Results of free vascularized fibular grafting for femoral head osteonecrosis in patients with systemic lupus erythematosus. Orthop. Clin. North Am. 35, 353–357 (2004).

    Article  PubMed  Google Scholar 

  97. Strand, V. Lessons learned from clinical trials in SLE. Autoimmun. Rev. 6, 209–214 (2007).

    Article  CAS  PubMed  Google Scholar 

  98. Jolly, M. How does quality of life of patients with systemic lupus erythematosus compare with that of other common chronic illnesses? J. Rheumatol. 32, 1706–1708 (2005).

    PubMed  Google Scholar 

  99. Falcini, F. & Nacci, F. Systemic lupus erythematosus in the young: the importance of a transition clinic. Lupus 16, 613–617 (2007).

    Article  CAS  PubMed  Google Scholar 

  100. Wagner-Weiner, L. Pediatric rheumatology for the adult rheumatologist. J. Clin. Rheumatol. 14, 109–119 (2008).

    Article  PubMed  Google Scholar 

  101. Lau, C. S. & Mak, A. The socioeconomic burden of SLE. Nat. Rev. Rheumatol. 5, 400–404 (2009).

    Article  PubMed  Google Scholar 

  102. Minden, K. et al. Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum. 46, 2392–2401 (2002).

    Article  PubMed  Google Scholar 

  103. Peterson, L. S., Mason, T., Nelson, A. M., O'Fallon, W. M. & Gabriel, S. E. Psychosocial outcomes and health status of adults who have had juvenile rheumatoid arthritis: a controlled, population-based study. Arthritis Rheum. 40, 2235–2240 (1997).

    Article  CAS  PubMed  Google Scholar 

  104. McElhone, K., Abbott, J. & Teh, L. S. A review of health related quality of life in systemic lupus erythematosus. Lupus 15, 633–643 (2006).

    Article  CAS  PubMed  Google Scholar 

  105. Ruperto, N. et al. Health-related quality of life in juvenile-onset systemic lupus erythematosus and its relationship to disease activity and damage. Arthritis Rheum. 51, 458–464 (2004).

    Article  PubMed  Google Scholar 

  106. Houghton, K. M., Tucker, L. B., Potts, J. E. & McKenzie, D. C. Fitness, fatigue, disease activity, and quality of life in pediatric lupus. Arthritis Rheum. 59, 537–545 (2008).

    Article  PubMed  Google Scholar 

  107. Moorthy, L. N. et al. Multicenter validation of a new quality of life measure in pediatric lupus. Arthritis Rheum. 57, 1165–1173 (2007).

    Article  PubMed  Google Scholar 

  108. Brunner, H. I. et al. Health-related quality of life and its relationship to patient disease course in childhood-onset systemic lupus erythematosus. J. Rheumatol. 36, 1536–1545 (2009).

    Article  PubMed  Google Scholar 

  109. Brunner, H. I. et al. Health of children with chronic arthritis: relationship of different measures and the quality of parent proxy reporting. Arthritis Rheum. 51, 763–773 (2004).

    Article  PubMed  Google Scholar 

  110. Moorthy, L. N. et al. Relationship between health-related quality of life and SLE activity and damage in children over time. Lupus 1 8, 622–629 (2009).

    Article  Google Scholar 

  111. Ravelli, A., Ruperto, N. & Martini, A. Outcome in juvenile onset systemic lupus erythematosus. Curr. Opin. Rheumatol. 17, 568–573 (2005).

    Article  PubMed  Google Scholar 

  112. Appenzeller, S., Marini, R. & Costallat, L. T. Damage did not independently influence mortality in childhood systemic lupus erythematosus. Rheumatol. Int. 25, 619–624 (2005).

    Article  PubMed  Google Scholar 

  113. Singh, S., Devidayal, K. L. & Joshi, K. Mortality patterns in childhood lupus—10 years' experience in a developing country. Clin. Rheumatol. 21, 462–465 (2002).

    Article  CAS  PubMed  Google Scholar 

  114. Olowu, W. Childhood-onset systemic lupus erythematosus. J. Natl Med. Assoc. 99, 777–784 (2007).

    PubMed  PubMed Central  Google Scholar 

  115. Uramoto, K. M. et al. Trends in the incidence and mortality of systemic lupus erythematosus, 1950–1992. Arthritis Rheum. 42, 46–50 (1999).

    Article  CAS  PubMed  Google Scholar 

  116. Mok, C. C., Ho, C. T., Wong, R. W. & Lau, C. S. Damage accrual in southern Chinese patients with systemic lupus erythematosus. J. Rheumatol. 30, 1513–1519 (2003).

    PubMed  Google Scholar 

  117. Pons-Estel, B. A. et al. The GLADEL multinational Latin American prospective inception cohort of 1,214 patients with systemic lupus erythematosus: ethnic and disease heterogeneity among “Hispanics”. Medicine (Baltimore) 83, 1–17 (2004).

    Article  Google Scholar 

  118. Durán, S. et al. Poverty, not ethnicity, accounts for the differential mortality rates among lupus patients of various ethnic groups. J. Natl Med. Assoc. 99, 1196–1198 (2007).

    PubMed  PubMed Central  Google Scholar 

  119. Danila, M. I. et al. Renal damage is the most important predictor of mortality within the damage index: data from LUMINA LXIV, a multiethnic US cohort. Rheumatology (Oxford) 48, 542–545 (2009).

    Article  Google Scholar 

  120. Ward, M. M., Pyun, E. & Studenski, S. Long-term survival in systemic lupus erythematosus. Patient characteristics associated with poorer outcomes. Arthritis Rheum. 38, 274–283 (1995).

    Article  CAS  PubMed  Google Scholar 

  121. Kasitanon, N., Magder, L. S. & Petri, M. Predictors of survival in systemic lupus erythematosus. Medicine (Baltimore) 85, 147–156 (2006).

    Article  Google Scholar 

  122. Manger, K. et al. Definition of risk factors for death, end stage renal disease, and thromboembolic events in a monocentric cohort of 338 patients with systemic lupus erythematosus. Ann. Rheum. Dis. 61, 1065–1070 (2002).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  123. Urowitz, M. B., Gladman, D. D., Tom, B. D., Ibañez, D. & Farewell, V. T. Changing patterns in mortality and disease outcomes for patients with systemic lupus erythematosus. J. Rheumatol. 35, 2152–2158 (2008).

    Article  PubMed  Google Scholar 

  124. Chambers, S. A., Allen, E., Rahman, A. & Isenberg, D. Damage and mortality in a group of British patients with systemic lupus erythematosus followed up for over 10 years. Rheumatology (Oxford) 48, 673–675 (2009).

    Article  Google Scholar 

Download references

Acknowledgements

S. Kamphuis is supported by the Sophia Children's Hospital Foundation and the Dutch Arthritis Foundation. She is an Assistant Professor in Rheumatology at the Sophia Children's Hospital, Erasmus Medical Center Rotterdam, Netherlands, currently on leave of absence. E. D. Silverman holds the Ho Family Chair in Pediatric Autoimmunity and is supported by grants from the Heart and Stroke Foundation of Ontario, Canada.

Author information

Authors and Affiliations

Authors

Contributions

S. Kamphuis and E. D. Silverman contributed equally to researching data for the article, discussing the content, writing the article, and reviewing and/or editing the manuscript before submission.

Corresponding author

Correspondence to Earl D. Silverman.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kamphuis, S., Silverman, E. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 6, 538–546 (2010). https://doi.org/10.1038/nrrheum.2010.121

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/nrrheum.2010.121

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing