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19.03.2020 | Original Article

Twelve-month outcome in juvenile proliferative lupus nephritis: results of the German registry study

Zeitschrift:
Pediatric Nephrology
Autoren:
Adriana Suhlrie, Imke Hennies, Jutta Gellermann, Anja Büscher, Peter Hoyer, Siegfried Waldegger, Simone Wygoda, Rolf Beetz, Bärbel Lange-Sperandio, Günter Klaus, Martin Konrad, Martin Holder, Hagen Staude, Wolfgang Rascher, Jun Oh, Lars Pape, Burkhard Tönshoff, Dieter Haffner, on behalf of the German Society of Paediatric Nephrology
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s00467-020-04501-x) contains supplementary material, which is available to authorized users.
This article is part of the Topical Collection on Pediatric Nephrology
This paper was presented at the 18th Congress of the International Pediatric Nephrology Association in Venice, Italy, in 2019.

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Abstract

Background

Children presenting with proliferative lupus nephritis (LN) are treated with intensified immunosuppressive protocols. Data on renal outcome and treatment toxicity is scare.

Methods

Twelve-month renal outcome and comorbidity were assessed in 79 predominantly Caucasian children with proliferative LN reported to the Lupus Nephritis Registry of the German Society of Paediatric Nephrology diagnosed between 1997 and 2015.

Results

At the time of diagnosis, median age was 13.7 (interquartile range 11.8–15.8) years; 86% showed WHO histology class IV, nephrotic range proteinuria was noted in 55%, and median estimated glomerular filtration rate amounted to 75 ml/min/1.73 m2. At 12 months, the percentage of patients with complete and partial remission was 38% and 41%, respectively. Six percent of patients were non-responders and 15% presented with renal flare. Nephrotic range proteinuria at the time of diagnosis was associated with inferior renal outcome (odds ratio 5.34, 95% confidence interval 1.26–22.62, p = 0.02), whereas all other variables including mode of immune-suppressive treatment (e.g., induction treatment with cyclophosphamide (IVCYC) versus mycophenolate mofetil (MMF)) were not significant correlates. Complications were reported in 80% of patients including glucocorticoid toxicity in 42% (Cushingoid appearance, striae distensae, cataract, or osteonecrosis), leukopenia in 37%, infection in 23%, and menstrual disorder in 20%. Growth impairment, more pronounced in boys than girls, was noted in 78% of patients.

Conclusions

In this cohort of juvenile proliferative LN, renal outcome at 12 months was good irrespectively if patients received induction treatment with MMF or IVCYC, but glucocorticoid toxicity was very high underscoring the need for corticoid sparing protocols.

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