Skip to main content

Advertisement

SpringerLink
Log in

Membranous glomerulonephritis: treatment response and outcome in children

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

The aim of this study was to characterize clinical features, treatment response, and outcome of idiopathic membranous glomerulonephritis (MGN) in a single-center cohort of children. A retrospective review of biopsy-proven idiopathic MGN in 12 children (mean age 11.9 years) was undertaken. Presentation was nephrotic syndrome (NS) (75%), hematuria/proteinuria (17%), and asymptomatic proteinuria (8%). Ten patients (83%) with NS and nephrotic range proteinuria (NRP) were treated with prednisone, and two patients with non-NRP were not treated with immunosuppressive medications. Steroid response in the treated patients was complete (10%), partial (40%), and absent (50%), respectively. Oral cyclophosphamide was used in seven patients of whom five were steroid resistant, one was steroid dependent, and one was partially responsive. At the mean follow up of 27 months, outcome parameters included an estimated glomerular filtration rate of 128 cc/min per 1.73 m2, albumin of 4.2 gm/dL, and a urine protein/creatinine ratio of 0.87 [median 0.16 (range 0.02–6.52)]. Remission was complete in 75% of the patients and partial in 17%. One patient (8%) with chronic kidney disease (stage 2) was unresponsive to therapy. Complete remission was significantly associated with the absence of chronic histological changes (p = 0.03). In conclusion, children with NS and/or NRP associated with MGN appear to have a good prognosis when treated with a combination of corticosteroids and cyclophosphamide.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Srivastava T, Simon SD, Alon US (1999) High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 13:13–18

    Article  CAS  Google Scholar 

  2. Churg J, Habib R, White RH (1970) Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. Lancet 760:1299–1302

    Article  CAS  Google Scholar 

  3. Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK, Singh U, Gupta A (2006) Steroid resistant nephrotic syndrome: role of histopathology. Indian Pediatr 43:55–60

    PubMed  Google Scholar 

  4. Ponticelli C, Zucchelli P, Passerini P, Cesana P, Locatelli F, Pasquali S, Sasdelli M, Redaelli B, Grassi C, Pozzi C, Bizzarri D, Banfi G (1995) A 10-year follow-up of a randomized study with methylprednisolone and chlorambucil in membranous nephropathy. Kidney Int 48:1600–1604

    Article  CAS  Google Scholar 

  5. Haas MMS, Karrison TG, Spargo BH (1997) Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976–1979 and 1995–1997. Am J Kidney Dis 30:621–631

    Article  CAS  Google Scholar 

  6. Schieppati A, Mosconi L, Perna A, Mecca G, Bertani T, Garattini S, Remuzzi G (1993) Prognosis of untreated patients with idiopathic membranous nephropathy. N Engl J Med 329:85–89

    Article  CAS  Google Scholar 

  7. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL (2001) Cyclosporine in patients with steroid-resistant membranous nephropathy: a randomized trial. Kidney Int 59:1484–1490

    Article  CAS  Google Scholar 

  8. Passerini P, Ponticelli C (2003) Corticosteroids, cyclophosphamide, and chlorambucil therapy of membranous nephropathy. Semin Nephrol 23:355–361

    Article  CAS  Google Scholar 

  9. Reichert LJ, Koene RA, Wetzels JF (1998) Prognostic factors in idiopathic membranous nephropathy. Am J Kidney Dis 31:1–11

    Article  CAS  Google Scholar 

  10. du Buf-Vereijken PW, Branten AJ, Wetzels JF (2005) Idiopathic membranous nephropathy: outline and rationale of a treatment strategy. Am J Kidney Dis 46:1012–1029

    Article  Google Scholar 

  11. Wu Q, Jinde K, Nishina M, Tanabe R, Endoh M, Okada Y, Sakai H, Kurokawa K (2001) Analysis of prognostic predictors in idiopathic membranous nephropathy. Am J Kidney Dis 37:380–387

    Article  CAS  Google Scholar 

  12. Perna A, Schieppati A, Zamora J, Giuliano GA, Braun N, Remuzzi G (2004) Immunosuppressive treatment for idiopathic membranous nephropathy: a systematic review. Am J Kidney Dis 44:385–401

    Article  Google Scholar 

  13. Olbing H, Greifer I, Bennett BP, Bernstein J, Spitzer A (1973) Idiopathic membranous nephropathy in children. Kidney Int 3:381–390

    Article  CAS  Google Scholar 

  14. Trainin EB, Boichis H, Spitzer A, Greifer I (1976) Idiopathic membranous nephropathy. Clinical course in children. N Y State J Med 76:357–360

    CAS  PubMed  Google Scholar 

  15. Ramirez F, Brouhard BH, Travis LB, Ellis EN (1982) Idiopathic membranous nephropathy in children. J Pediatr 101:677–681

    Article  CAS  Google Scholar 

  16. Lee BH, Cho HY, Kang HG, Ha IS, Cheong HI, Moon KC, Lim IS, Choi Y (2006) Idiopathic membranous nephropathy in children. Pediatr Nephrol 21:1707–1715

    Article  Google Scholar 

  17. Jennette JC, Iskandar SS, Dalldorf FG (1983) Pathologic differentiation between lupus and nonlupus membranous glomerulopathy. Kidney Int 24:377–385

    Article  CAS  Google Scholar 

  18. Atta MG, Choi MJ, Longenecker JC, Haymart M, Wu J, Nagajothi N, Racusen LC, Scheel PJ, Brancati FL, Fine DM (2005) Nephrotic range proteinuria and CD4 count as noninvasive indicators of HIV-associated nephropathy. Am J Med 118:1288

    Article  Google Scholar 

  19. National High Blood Pressure Education Program Work Group on Hypertension Control in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576

    Article  Google Scholar 

  20. Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 34:571–590

    Article  CAS  Google Scholar 

  21. White RH, Glasgow EF, Mills RJ (1970) Clinicopathological study of nephrotic syndrome in childhood. Lancet 1:1353–1359

    Article  CAS  Google Scholar 

  22. The Southwest Pediatric Nephrology Study Group (1986) Comparison of idiopathic and systemic lupus erythematosus-associated membranous glomerulonephropathy in children. Am J Kidney Dis 7:115–124

    Article  Google Scholar 

  23. Yoshikawa N, Ito H, Yamada Y, Hashimoto H, Katayama Y, Matsuyama S, Hasegawa O, Okada S, Hajikano H, Yoshizawa H, Mayumi M, Matsuo T (1985) Membranous glomerulonephritis associated with hepatitis B antigen in children: a comparison with idiopathic membranous glomerulonephritis. Clin Nephrol 23:28–34

    CAS  PubMed  Google Scholar 

  24. Cameron JS (1990) Membranous nephropathy in childhood and its treatment. Pediatr Nephrol 4:193–198

    Article  CAS  Google Scholar 

  25. Kallen RJ, Lee SK, Aronson AJ, Spargo BH (1977) Idiopathic membranous glomerulopathy preceding the emergence of systemic lupus erythematosus in two children. J Pediatr 90:72–76

    Article  CAS  Google Scholar 

  26. Wakai S, Magil AB (1992) Focal glomerulosclerosis in idiopathic membranous glomerulosclerosis. Kidney Int 41:428–434

    Article  CAS  Google Scholar 

  27. Troyanov S, Wall CA, Miller JA, Scholey JW, Cattran DC (2004) Idiopathic membranous nephropathy: definition and relevance of a partial remission. Kidney Int 66:1199–1205

    Article  Google Scholar 

  28. Filler G, Young E, Geier P, Carpenter B, Drukker A, Feber J (2003) Is there really an increase in non-minimal change nephrotic syndrome in children? Am J Kidney Dis 42:1107–1113

    Article  Google Scholar 

  29. Latham P, Poucell S, Koresaar A, Arbus G, Baumal R (1982) Idiopathic membranous glomerulopathy in Canadian children: a clinicopathologic study. J Pediatr 101:682–685

    Article  CAS  Google Scholar 

  30. Ruggenenti P, Mosconi L, Vendramin G, Moriggi M, Remuzzi A, Sangalli F, Remuzzi G (2000) ACE inhibition improves glomerular size selectivity in patients with idiopathic membranous nephropathy and persistent nephrotic syndrome. Am J Kidney Dis 35:381–391

    Article  CAS  Google Scholar 

  31. The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia) (1997) Randomised placebo-controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non-diabetic nephropathy. Lancet 349:1857–1863

    Article  Google Scholar 

  32. Tsukahara H, Takahashi Y, Yoshimoto M, Hayashi S, Fujisawa S, Suehiro F, Akaishi K, Nomura Y, Morikawa K, Sudo M (1993) Clinical course and outcome of idiopathic membranous nephropathy in Japanese children. Pediatr Nephrol 7:387–391

    Article  CAS  Google Scholar 

  33. Habib R, Kleinknecht C, Gubler MC (1973) Extramembranous glomerulonephritis in children: report of 50 cases. J Pediatr 82:754–766

    Article  CAS  Google Scholar 

  34. Halevy D, Radhakrishnan J, Appel GB (2001) Racial and socioeconomic factors in glomerular disease. Semin Nephrol 21:403–410

    Article  CAS  Google Scholar 

  35. Trompeter RS, Evans PR, Barratt TM (1981) Gonadal function in boys with steroid-responsive nephrotic syndrome treated with cyclophosphamide for short periods. Lancet 1:1177–1179

    Article  CAS  Google Scholar 

  36. Alexopoulos E, Papagianni A, Tsamelashvili M, Leontsini M, Memmos D (2006) Induction and long-term treatment with cyclosporine in membranous nephropathy with the nephrotic syndrome. Nephrol Dial Transplant 21:3127–3132

    Article  CAS  Google Scholar 

  37. Ponticelli C, Altieri P, Scolari F, Passerini P, Roccatello D, Cesana B, Melis P, Valzorio B, Sasdelli M, Pasquali S, Pozzi C, Piccoli G, Lupo A, Segagni S, Antonucci F, Dugo M, Minari M, Scalia A, Pedrini L, Pisano G, Grassi C, Farina M, Bellazzi R (1998) A randomized study comparing methylprednisolone plus chlorambucil versus methylprednisolone plus cyclophosphamide in idiopathic membranous nephropathy. J Am Soc Nephrol 9:444–450

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. The Carman and Ann Adams Department of Pediatrics, Division of Nephrology, Children’s Hospital of Michigan, Wayne State University School of Medicine, 3901 Beaubien Blvd, Detroit, MI, 48201-2196, USA

    Rudolph P. Valentini, Tej K. Mattoo & Gaurav Kapur

  2. Akron Children’s Hospital, Department of Nephrology, Northeastern Ohio Universities College of Medicine, Akron, OH, USA

    Abubakr Imam

Authors
  1. Rudolph P. Valentini
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tej K. Mattoo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gaurav Kapur
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Abubakr Imam
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rudolph P. Valentini.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Valentini, R.P., Mattoo, T.K., Kapur, G. et al. Membranous glomerulonephritis: treatment response and outcome in children. Pediatr Nephrol 24, 301–308 (2009). https://doi.org/10.1007/s00467-008-1005-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-008-1005-9

Keywords

Search

Navigation