Abstract
We report a multicenter study of Chinese children in Hong Kong with systemic lupus erythematosus (SLE) nephritis. Children were included if: they fulfilled the ACR criteria, had significant proteinuria or casturia, were Chinese and younger than 19 years and had been diagnosed with SLE between January 1990 and December 2003. Investigators in each center retrieved data on clinical features, biopsy reports, treatment and outcome of these patients. There were 128 patients (eight boys, 120 girls; mean age: 11.9±2.8 years). About 50% presented with multisystem illness and 40% with nephritic/nephrotic symptoms. Negative anti-dsDNA antibodies were found in 6% of the patients. Renal biopsy revealed WHO Class II, III, IV and V nephritis in 13 (10%), 22 (17%), 69 (54%) and 13 (10%) patients, respectively. The clinical severity of the nephritis did not accurately predict renal biopsy findings. The follow-up period ranged from 1 to 16.5 years (mean±SD: 5.76±3.61 years). During the study five patients died (two from lupus flare, one from cardiomyopathy, two from infections). Four patients had endstage renal failure (ESRF) (one died during a lupus flare). All deaths and end-stage renal failure occurred in the Class IV nephritis group. Chronic organ damage was infrequent in the survivors. The actuarial patient survival rates at 5, 10 and 15 years of age were 95.3, 91.8, and 91.8%, respectively. For Class IV nephritis patients, the survival rates without ESRF at 5, 10, and 15 years were 91.5, 82.3 and 76%, respectively. The survival and chronic morbidity rates of the Chinese SLE children in the present study are comparable to those of other published studies.
Similar content being viewed by others
References
Stichweh D, Arce E, Pascual V (2004) Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol 16:577–587
Carreno L, Lopez-Longo FJ, Monteagudo I, Rodriguez-Mahou M, Bascones M, Gonzalez CM, Saint-Cyr C, Lapointe N (1999) Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus 8:287–292
Perfumo F, Martini A (2005) Lupus nephritis in children. Lupus 14:83–88
Meislin AG, Rothfield N (1968) Systemic lupus erythematosus in childhood. Analysis of 42 cases, with comparative data on 200 adult cases followed concurrently. Pediatrics 42:37–49
Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S, Garcia-Carrasco M, Seisdedos L, Ingelmo M (1998) 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
Rood MJ, ten Cate R, van Suijlekom-Smit LWA, de Ouden EJ, Ouwerkerk FE, Breedveld FC, Huizinga TWJ (1999) Childhood-onset systemic lupus erythematosus. Clinical presentation and prognosis in 31 patients. Scand J Rheumatol 28:222–226
Alarcon GS, McGwin Jr G, Bartolucci AA, Roseman J, Lisse J, Fessler BJ, Bastian HM, Friedman AW, Reveille JD, LUMINA Study Group. Lupus in Minority Populations, Nature versus Nature (2001) Systemic lupus erythematosus in three ethnic groups. IX. Differences in damage accrual. Arthritis Rheum 44:2797–2806
Abu-Shakra M, Urowitz MB, Gladman DD, Gough J (1995) Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death. J Rheumatol 22:1259–1264
Tucker LB, Menon S, Schaller JG, Isenberg DA (1995) Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 34:866–872
Mok CC, Wong RWS, Lau CS (1999) Lupus nephritis in southern Chinese patients: clinicopathologic findings and long-term outcome. Am J Kidney Dis 34:315–323
Mok CC, Lee KW, Ho CTK, Lau CS, Wong RWS (2000) A prospective study of survival and prognostic indicators of systemic lupus erythematosus in a southern Chinese population. Rheumatology 39:399–406
Mok CC, Lau CS (2005) Lupus in Hong Kong Chinese. Lupus 12:717–722
Yang LY, Chen WP, Lin CY (1994) Lupus Nephritis in Children – A Review of 167 Patients. Pediatrics 94:335–340
Churg J, Sobin LH (1981) Renal disease: classification and atlas of glomerular diseases. Igaku-Shoin, Tokyo
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH (1992) Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 35:630–640
Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, Bacon P, Bombardieri S, Hanly J, Hay E, Isenberg D, Jones J, Kalunian K, Maddison P, Nived O, Petri M, Richter M, Sanchez-Guerrero J, Snaith M, Sturfelt G, Symmons D, Zoma A (1996) 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
Brunner HI, Silverman ED, To T, Bombardier C, Feldman BM (2002) Risk factors for damage in childhood-onset systemic lupus erythematosus. Cumulative disease activity and medication use predict disease damage. Arthritis Rheum 46:436–444
King KK, Kornreich HK, Bernstein BH, Singsen BH, Hanson V (1977) The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum 20:287–294
Vilar MJP, Sato EI (2002) Estimating the incidence of systemic lupus erythematosus in a tropical region (Natal, Brazil). Lupus 11:528–532
Cassidy JT, Petty RE (2001) Systemic Lupus Erythematosus. In: Cassidy JT, Petty RE (eds) Textbook of Pediatric Rheumatology. WB Saunders, Philadelphia, pp 396–449
Brunner HI, Freedman M, Silverman ED (1999) Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis Rheum 42:2346–2355
Swaak AJG, van den Brink HG, Smeenk RJT, Manger K, Kalden JR, Tosi S, Marchesoni A, Domljan Z, Rozman B, Logar D, Pokorny G, Kovacs L, Kovacs A, Vlachoyiannopoulos PG, Moutsopoulos HM, Chwalinska-Sadowska H, Dratwianka B, Kiss E, Cikes N, Branimir A, Schneider M, Fischer R, Bombardieri S, Mosca M, Graninger W, Smolen JS (1999) Systemic lupus erythematosus: clinical features in patients with a disease duration of over 10 years, first evaluation. Rheumatology 38:953–958
Levine JS, Branch DW, Rauch J (2002) The antiphospholipid syndrome. N Engl J Med 346:752–763
Houssiau FA (2004) Management of lupus nephritis: an update. J Am Soc Nephrol 15:2694–2704
Hagelberg S, Lee Y, Bargman J, Mah G, Schneider R, Laskin C, Eddy A, Gladman D, Urowitz M, Hebert D, Silverman E (2002) Longterm followup of childhood lupus nephritis. J Rheumatol 29:2635–2642
Baqi N, Moazami S, Singh A, Ahmad H, Balachandra S, Tejani A (1996) Lupus nephritis in children: a longitudinal study of prognostic factors and therapy. J Am Soc Nephrol 7:924–929
Niaudet P (2000) Treatment of lupus nephritis in children. Pediatr Nephrol 14:158–166
Mok CC, Ho CTK, Siu YP, Chan KW, Kwan TH, Lau CS, Wong RWS, Au TC (2002) Treatment of diffuse proliferative lupus glomerulonephritis: a comparison of two cyclophosphamide-containing regimens. Am J Kidney Dis 38:256–264
McCurdy DK, Lehman TJA, Bernstein B, Hanson V, King KK, Nadorra R, Landing BH (1992) Lupus nephritis: prognostic factors in children. Pediatrics 89:240–246
Emre S, Bilge I, Sirin A, Kilicaslan I, Nayir A, Oktem F, Uysal V (2001) Lupus Nephritis in children: prognostic significance of clinicopathological findings. Nephron 87:118–126
Bogdanovic R, Nikolic V, Pasic S, Dimitrijevic J, Lipkovska-Markovic J, Eric-Marinkovic J, Ognjanovic M, Minic A, Stajic N (2004) Lupus nephritis in childhood: a review of 53 patients followed at a single center. Pediatr Nephrol 19:36–44
Miettunen PM, Ortiz-Alvarez O, Petty RE, Cimaz R, Malleson PN, Cabral DA, Ensworth S, Tucker LB (2004) Gender and ethnic origin have no effect on longterm outcome of childhood-onset systemic lupus erythematosus. J Rheumatol 31:1650–1654
Acknowledgements
We would like to thank the chiefs-of-service of all pediatric departments in the Hong Kong Hospital Authority for their support and permission to allow us to review the clinical data of the patients in their departments.
Author information
Authors and Affiliations
Corresponding author
Additional information
An erratum to this article can be found at https://doi.org/10.1007/s00467-006-0190-7
Rights and permissions
About this article
Cite this article
Wong, SN., Tse, KC., Lee, TL. et al. Lupus nephritis in Chinese children – a territory-wide cohort study in Hong Kong. Pediatr Nephrol 21, 1104–1112 (2006). https://doi.org/10.1007/s00467-006-0052-3
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-006-0052-3