Abstract
We retrospectively reviewed the cases of 13 lupus nephritis children with pure membranous glomerulonephritis (MGN; Group A) and ten children with mixed proliferative and membranous nephritis (Group B). The children were identified through a territory-wide survey of patients between 1990 and 2003. All were ethnic Chinese. Age at diagnosis ranged from 3.7 to 18.6 years (Group A) and from 9.6 to 22.1 years (Group B). Female-to-male ratios were 12:1 (Group A) and 9:1 (Group B). Group A patients were more often nephrotic than Group B patients (11/13 vs. 5/10, p = 0.17). The glomerular filtration rate (GFR) at presentation was normal in all but two patients (one from each group). For induction, Group B patients consistently received prednisolone and cyclophosphamide; in contrast, the cytotoxic regimens in Group A patients varied from cyclophosphamide (five patients), mycophenolate mofetil (two patients), azathiorpine plus cyclosporine (one patient), and azathioprine alone (one patient). After a median follow-up of 7.6–7.8 years, one Group A patient had died of fulminant lupus. One survivor in Group B had a GFR < 90 ml/min per 1.73 m2. Proteinuria persisted in five Group A patients and two Group B patients. In conclusion, Group B patients had good prognosis in terms of survival and proteinuria control. The only death occurred in Group A, and five of the 12 survivors in this group had persistent proteinuria. Further studies are needed to define the best treatment for pure lupus MGN.
Similar content being viewed by others
References
Austin HA, Illei GG (2005) Membranous lupus nephritis. Lupus 14:65–71
Cameron JS (1994) Lupus nephritis in childhood and adolescence. Pediatr Nephrol 8:230–249
Chan TM, Li FK, Hao WK, Lui SF, Chan KW, Tang S, Lai KN (1999) Treatment of membranous lupus nephritis with nephrotic syndrome by sequential immunosuppression. Lupus 8:545–551
Sloan RP, Schwartz MM, Korbet SM, Borok RZ (1996) Long-term outcome in systemic lupus erythematosus membranous glomerulonephritis. J Am Soc Nephrol 7:299–305
Moroni G, Maccario M, Banfi G, Quaglini S, Ponticelli C (1998) Treatment of membranous lupus nephritis. Am J Kidney Dis 31:681–686
Mok CC, Ying KY, Lau CS, Yim CW, Ng WL, Wong WS, Au TC (2004) Treatment of pure membranous lupus nephropathy with prednisone and azathioprine: an open-label trial. Am J Kidney Dis 43:269–276
Tam LS, Li EK, Szeto CC, Wong SM, Leung CB, Lai FM, Wong KC, Lui SF (2001) Treatment of membranous lupus nephritis with prednisone, azathioprine and cyclosporin A. Lupus 10:827–829
Hallegua D, Wallace DJ, Metzger AL, Rinaldi RZ, Klinenberg JR (2000) Cyclosporine for lupus membranous nephritis: Experience with ten patients and review of the literature. Lupus 9:241–251
Borba EF, Guedes LK, Christmann RB, Figueiredo CP, Goncalves CR, Bonfa E (2006) Mycophenolate mofetil is effective in reducing lupus glomerulonephritis proteinuria. Rheumatol Int 26:1078–1083
Spetie DN, Tang Y, Rovin BH, Nadasdy T, Nadasdy G, Pesavento TE, Hebert LA (2004) Mycophenolate therapy of SLE membranous nephropathy. Kidney Int 66:2411–2415
Mercadal L, Montcel ST, Nochy D, Queffeulou G, Piette JC, Isnard-Bagnis C, Martinez F, the Groupe d'Etudes Nephrologiques en Ile de France (2002) Factors affecting outcome and prognosis in membranous lupus nephropathy. Nephrol Dial Transplant 17:1771–1778
Al Salloum AA (2003) Cyclophosphamide therapy for lupus nephritis: poor renal survival in Arab children. Pediatr Nephrol 18:357–361
Zappitelli M, Duffy C, Bernard C, Scuccimarri R, Duffy KW, Kagan R, Gupta IR (2004) Clinicopathological study of the WHO classification in childhood lupus nephritis. Pediatr Nephrol 19:503–510
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
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
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
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
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
Nathanson S, Salomon R, Ranchin B, Macher MA, Lavocat MP, Krier MJ, Baudouin V, Azema C, Bader-Meunier B, Deschenes G (2006) Prognosis of lupus membranous nephropathy in children. Pediatr Nephrol 21:1113–1116
Cramer CH2nd, Mills M, Valentini RP, Smoyer WE, Haftel H, Brophy PD (2007) Clinical presentation and outcome in a cohort of paediatric patients with membranous lupus nephritis. Nephrol Dial Transplant 22:3495–3500
Lau KK, Jones DP, Ault BH (2007) Prognosis of lupus membranous nephritis in children. Lupus 16:70
Churg J, Sobin LH (1982) Lupus nephritis. In: Churg J, Sobin LH (eds) Renal disease: classification and atlas of glomerular diseases. Igaku-Shoin, New York, pp 127–149
Churg J, Bernstein J, Glassock RJ (1995) Lupus nephritis. In: Churg J, Bernstein J, Glassock RJ (eds) Renal disease: classification and atlas of glomerular diseases. Igaku-Shoin, New York, pp 151–179
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M, International Society of Nephrology and Renal Pathology Society Working Group on the Classification of Lupus Nephritis (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 65:521–530
Acknowledgements
We would like to thank the chiefs-of-service of the participating paediatric departments for their support and their extending their approval for us to review the clinical data of their patients.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Wong, SN., Chan, W.KY., Hui, J. et al. Membranous lupus nephritis in Chinese children—a case series and review of the literature. Pediatr Nephrol 24, 1989–1996 (2009). https://doi.org/10.1007/s00467-009-1257-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-009-1257-z