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15.02.2017 | Original Article

Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents

verfasst von: Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski

Erschienen in: Pediatric Nephrology | Ausgabe 7/2017

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Abstract

Background

Henoch–Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch–Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis.

Methods

All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files. The primary endpoint was proteinuria remission defined as a proteinuria <200 mg/L.

Results

Ninety-two children were included in the study with a median follow-up of 36 (6–120) months; 28% had nephrotic syndrome, 31% proteinuria >3 g/L, 52% proteinuria between 1 and 3 g/L, and 18% proteinuria <1 g/L. Forty-seven percent of patients received orally treatment with steroids alone, 37% received methylprednisolone pulses followed by steroids orally, 18% received no steroids. Although 85% reached remission during follow-up, 12% did not maintain complete remission over time so that only 75% remained in complete remission by the end of the follow-up. Univariate analysis found a higher likelihood of remission in patients with higher proteinuria at disease onset (p = 0.009). This trend was not found in the multivariate analysis after adjusting for treatments, as patients with higher proteinuria were most often treated with steroids.

Conclusion

Our study shows that one fourth of patients with HSPN class 2 remain proteinuric and thus carry the risk of developing chronic kidney disease over the long term. This finding, together with the better outcome of patients treated with steroids, is in favor of using high-dose steroids orally or IV in these patients.

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Titel: Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents
verfasst von: Jean Daniel Delbet
Julien Hogan
Bilal Aoun
Iulia Stoica
Rémi Salomon
Stéphane Decramer
Isabelle Brocheriou
Georges Deschênes
Tim Ulinski
Publikationsdatum: 15.02.2017
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 7/2017
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-017-3604-9

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Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents