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Published in: Pediatric Nephrology 3/2013

01-03-2013 | Brief Report

Late recovery of renal function by rituximab in a patient with Wegener’s granulomatosis

Authors: Michal Malina, Betti Schaefer, Rüdiger Waldherr, Elke Wühl, Franz Schaefer, Claus Peter Schmitt

Published in: Pediatric Nephrology | Issue 3/2013

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Abstract

Background

Rituximab has proven effective in the treatment of complicated granulomatosis with polyangiitis (Wegener’s, GPA). Two controlled trials in adults demonstrated beneficial effects of rituximab compared to cyclophosphamide in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis to induce remission and to treat relapses. Pediatric experience with rituximab in GPA is limited; the impact on renal function is unknown.

Case-diagnosis/treatment

We report a female adolescent with GPA and necrotizing glomerulonephritis that lead to end-stage renal disease (ESRD). After 22 months of peritoneal dialysis, she still experienced relapses and major treatment-associated adverse effects. After a single dose of rituximab, she rapidly achieved clinical remission and, unexpectedly, steadily recovered glomerular filtration rate, plateauing at 25 ml/min/1.73 m2. Peritoneal dialysis could be discontinued for 16 months.

Conclusions

This case documents a potent beneficial effect of rituximab on renal manifestation of GPA even in long-established ESRD.
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Metadata
Title
Late recovery of renal function by rituximab in a patient with Wegener’s granulomatosis
Authors
Michal Malina
Betti Schaefer
Rüdiger Waldherr
Elke Wühl
Franz Schaefer
Claus Peter Schmitt
Publication date
01-03-2013
Publisher
Springer-Verlag
Published in
Pediatric Nephrology / Issue 3/2013
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-012-2332-4

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