Levamisole and ANCA positivity in childhood nephrotic syndrome

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

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.

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.

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.