Protracted remission of proteinuria after combined therapy with plasmapheresis and anti-CD20 antibodies/cyclophosphamide in a child with oligoclonal IgM and glomerulosclerosis

Gian Marco Ghiggeri, Luca Musante, Giovanni Candiano, Maurizio Bruschi, Laura Santucci, Giancarlo Barbano, Antonella Trivelli, Lucia Rivabella, Rosanna Gusmano, Francesco Perfumo

Research output: Contribution to journalArticle

Abstract

We describe a child presenting with oligoclonal plasma IgM (1.2 g%) and nephrotic syndrome with focal segmental glomerulosclerosis. Oligoclonality was demonstrated by the analysis of the complementary determining region 3 (CDR 3) on immunoglobulin heavy chains and by two dimensional electrophoresis and Western blot analysis that showed the bulk of isoforms having a cationic μU chain compared with the normal homologue (pI 7.5 vs 6.5). Urinary light chains were absent, and bone marrow aspirate was normal. Usual therapies for nephrotic syndrome with steroids and cyclosporin were useless. At the age of 9 years the patient was treated with plasmapheresis plus cyclophosphamide (2 mg/kg per day for 60 days), which temporarily reduced plasma IgM, and proteinuria was normal for 3 years. After this period, due to new recurrence of nephrotic syndrome, the patient received a cycle with anti-CD20 antibodies (500 mg/m2 every week for a month) associated with a cycle of plasmapheresis that normalized proteinuria again, and, after 3 years, the proteinuria is still in remission. This is the first case of nephrotic syndrome associated with oligoclonal plasma IgM and mesangial IgM deposits. Both cyclophosphamide and anti-CD20 antibodies associated with plasmapheresis induced, at different stages, stable and protracted remission of proteinuria without evident side effects. Long term efficacy and safety of the association are still to be determined.

Original languageEnglish
Pages (from-to)1953-1956
Number of pages4
JournalPediatric Nephrology
Volume22
Issue number11
DOIs
Publication statusPublished - Nov 2007

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Keywords

  • Cationic IgM
  • Focal glomerulosclerosis
  • Mesangial proliferative glomerulonephritis
  • Nephrotic syndrome

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

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