Membranous lupus nephritis in Chinese children--a case series and review of the literature

Pediatr Nephrol. 2009 Oct;24(10):1989-96. doi: 10.1007/s00467-009-1257-z. Epub 2009 Jul 22.

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 m(2). 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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Anti-Inflammatory Agents / therapeutic use
  • Asian Continental Ancestry Group
  • Azathioprine / therapeutic use
  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use
  • Cyclosporins / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Glomerular Filtration Rate
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lupus Nephritis / drug therapy*
  • Lupus Nephritis / physiopathology
  • Male
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Prednisolone / therapeutic use
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Cyclosporins
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisolone
  • Mycophenolic Acid
  • Azathioprine