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Levamisole and ANCA positivity in childhood nephrotic syndrome

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Abstract

Background

This study aimed to determine the prevalence of ANCA positivity in children managed with levamisole as a steroid-sparing agent for nephrotic syndrome (NS).

Methods

Medical records of children with steroid-sensitive NS managed with levamisole therapy at Sydney Children’s Hospital between 1/1/2000 and 31/12/2018 were retrospectively reviewed. Main outcome measure was side effects of levamisole therapy including ANCA positivity.

Results

Seventy-one children, median age 3 years and 1 month (IQR 29–68 months) at first presentation, were subsequently managed with levamisole. 60.6% were male and 65% Caucasian. 47.9% had frequently relapsing (FR)NS and 52.1% steroid-dependent (SD)NS. Overall, there was a median reduction in relapses from 3 (IQR 1–5) to 0.4 relapses (IQR 0–1) per year after levamisole was commenced. Levamisole was successful in preventing relapse in 19 (29%) patients and was used for median 24 (22 to 25) months. Levamisole was discontinued due to relapse in 25 patients (38%) after median 12 (5–28) months. Side effects occurred in 28 patients (42.4%); the most common side effect was ANCA positivity in 12 patients. In eleven of these patients, levamisole was discontinued; in one patient, low-level titres were documented and spontaneously resolved without cessation of levamisole. Two patients developed ANCA-associated vasculitis.

Conclusion

ANCA positivity is a common side effect of levamisole and was seen in 18.2% of our patients. Monitoring is required to determine side effects including ANCA positivity and treatment modified accordingly.

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Data availability

The datasets generated and analysed during the current study are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Nephrology, Sydney Children’s Hospital, Randwick, NSW, Australia

    Leah Krischock & Sean E. Kennedy

  2. School of Women’s and Children’s Health, UNSW Medicine, University of New South Wales, Sydney, NSW, Australia

    Leah Krischock, Pasan Pannila & Sean E. Kennedy

Authors
  1. Leah Krischock
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  2. Pasan Pannila
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  3. Sean E. Kennedy
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Contributions

L Krischock and S Kennedy contributed to the study design. Data collection and analysis were performed by L Krischock and P Pannila. All authors contributed to interpretation of findings. Initial draft of the manuscript was written by L Krischock. S Kennedy commented on previous versions of the manuscript. L Krischock and S Kennedy read and approved the final manuscript.

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Correspondence to Leah Krischock.

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The authors declare that they have no conflict of interest.

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Approved by the Human Research Ethics Committee at Sydney Children’s Hospital, Randwick (LNR/15/SCHN/503).

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Krischock, L., Pannila, P. & Kennedy, S.E. Levamisole and ANCA positivity in childhood nephrotic syndrome. Pediatr Nephrol 36, 1795–1802 (2021). https://doi.org/10.1007/s00467-020-04915-7

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  • DOI: https://doi.org/10.1007/s00467-020-04915-7

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