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

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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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Acknowledgments

We thank the colleagues of the participating centers and the study nurses from Hannover Medical School for data collection and registry support.

Funding

This study was supported by a grant of €5,000 by the German Society of Paediatric Nephrology. This had no influence on analysis content.

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Contributions

D.H. and I.H. designed the study; A.S. structured the database and analyzed the data. A.S., I.H., and D.H. drafted and revised the paper; all other authors recruited patients to this registry and approved the final version of the manuscript.

Corresponding author

Correspondence to Dieter Haffner.

Ethics declarations

Written informed consent was obtained from all parents/guardians to participate in the registry, with assent from patients when appropriate for their age. The study complies with the declaration of Helsinki and was approved by the ethics committee of the Medical University of Rostock, Germany.

Disclaimer

The results in this article have not been published previously in whole or in part, except in abstract form.

Conflict of interest

D.H. has received research grants from Sandoz, Kyowa Kirin, Horizon, and Amgen and has received speaker and/or consultant fees from Amgen, Sandoz, Kyowa Kirn, Pfizer, Merck Serono, Horizon, and Chiesi. All the other authors declare that they have no conflict of interest.

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Suhlrie, A., Hennies, I., Gellermann, J. et al. Twelve-month outcome in juvenile proliferative lupus nephritis: results of the German registry study. Pediatr Nephrol 35, 1235–1246 (2020). https://doi.org/10.1007/s00467-020-04501-x

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