Research article

Idiopathic membranous nephropathy in pediatric patients: presentation, response to therapy, and long-term outcome

Ashton Chen¹ , Rachel Frank¹ , Suzanne Vento¹ , Virginia Crosby¹ , Manju Chandra¹ , Bernard Gauthier¹ , Elsa Valderrama² and Howard Trachtman¹

¹Department of Pediatrics, Division of Nephrology, Schneider Children's Hospital of North Shore-Long Island Jewish Health System, New Hyde Park, NY 11040-1432, USA

²Department of Pathology, Schneider Children's Hospital of North Shore-Long Island Jewish Health System, New Hyde Park, NY 11040-1432, USA

author email corresponding author email

BMC Nephrology 2007, 8:11doi:10.1186/1471-2369-8-11

Published:	6 August 2007

Abstract

Background

Idiopathic membranous nephropathy (IMN) is one of the most common causes of primary nephrotic syndrome in adults. However, it is a relatively rare entity in the pediatric population and there is a paucity of data about the incidence, prognosis, and optimal treatment of IMN in children and adolescents. We conducted this study to evaluate pediatric patients with IMN in order to clarify the presentation, response to therapy, and clinical outcome.

Methods

A retrospective chart review was performed on patients identified with biopsy-proven IMN between 1988–2005. Patients with systemic lupus erythematosus or hepatitis-related lesions were excluded. The following data were tabulated: age, gender, ethnicity, presenting clinical and laboratory findings, proteinuria in a first morning urine specimen, estimated glomerular filtration rate (GFR_e), histopathology, type and duration of treatment, and clinical status at final evaluation.

Results

13 cases of IMN were identified out of 460 renal biopsies performed for evaluation of primary kidney disease during the study interval. Mean age was 9.6 ± 4.6, gender 6 M:7 F, ethnicity 8 W:2 B:3 H. At the initial visit hematuria was present in 9 patients, edema in 5, nephrotic-range proteinuria in 5, and hypertension in 3. Mean urinary protein:creatinine ratio 3.3 ± 2.5 and all patients had a normal GFR_e. Classic glomerular findings of IMN were seen in all renal specimens, with concomitant interstitial changes in 2 cases. Treatment included an angiotensin converting enzyme inhibitor or angiotensin receptor blocker in 11 cases. Most patients were also given immunosuppressive medications – prednisone in 10, a calcineurin inhibitor in 5, and mycophenolate mofetil or azathioprine in 3 patients. At the last follow-up, 42 ± 35 months after the diagnostic biopsy, 7 children were hypertensive and the urine protein:creatinine ratio was 2.3 ± 3.1. The mean GFR_e was 127 ± 57 mL/min/m². Three patients had Chronic Kidney Disease Stage 3, all of whom were also hypertensive.

Conclusion

IMN is a rare but serious glomerulopathy in pediatrics. We estimate that it accounts for approximately 3% of renal biopsies. Long-term prognosis is guarded because approximately 50% of patients may have evidence of progressive kidney disease.