Abstract
Background
Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning.
Methods
Observational cross-sectional study wherein children aged 2 to 18 years with frequently relapsing/steroid dependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12 months were tested for ANCA.
Results
A total of 210 children (33% female), median age of 7.3 (IQR: 5.6–9.6) years, and a median duration of levamisole exposure of 21 (IQR: 15–30) months were tested. ANCA was positive in 18% (n = 37): 89% (n = 33) perinuclear ANCA (pANCA), 3% (n = 1) cytoplasmic ANCA (cANCA), and 8% (n = 3) both. Of ANCA-positive children, none had reduced eGFR or abnormal urinalysis. The majority of these children were asymptomatic (81%, n = 30). Rash was more common among ANCA-positive children [6/37 (16%) vs. 3/173 (2%), p = 0.0001]. On multivariate analysis, higher age (OR = 1.02, [95th CI: 1.01 to 1.03], p = 0.007) and longer duration of levamisole exposure (OR = 1.05, [95th CI: 1.02 to 1.08], p = 0.0007) were associated with ANCA positivity. Levamisole was stopped in ANCA-positive children with the resolution of any clinical manifestations if present. Repeat ANCA testing was performed in 54% (20/37), and all were ANCA negative by 18 months.
Conclusions
Children with FRNS/SDNS on longer duration of levamisole were associated with increasing prevalence of ANCA positivity, but most of these children were clinically asymptomatic. Prospective studies are required to determine the chronology of ANCA positivity and its clinical implication.
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Data availability
Data regarding this study is kept at the ICH repository and will be available on request to the ICH research committee and its clearance (email chrac23@gmail.com).
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Acknowledgements
Ms. Adrita Saha, Departmental Coordinator, Institute of Child Health, Kolkata; Dr. Ashish Pundhir, Department of Community Medicine/Family Medicine, All India Institute of Medical Sciences, Kalyani.
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RS and SS: conceptualized, designed, collected data, analyzed data, and wrote the draft; SB, SA, SP, DD, JS, MM, and AP: helped in data collection and analysis and gave critical input while writing; YT helped with drafting, critical review, and data analysis.
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Sinha, R., Sarkar, S., Banerjee, S. et al. Antineutrophil cytoplasmic antibody in children with nephrotic syndrome treated with levamisole: a cross-sectional cohort study. Pediatr Nephrol (2024). https://doi.org/10.1007/s00467-024-06362-0
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DOI: https://doi.org/10.1007/s00467-024-06362-0