Progress in immunosuppression and increased medication adherence have improved the outlook of patients with childhood-onset lupus nephritis. Nonetheless, the disease is associated with adverse long-term outcomes, especially among patients with risk factors, a new Chinese study concludes.
Investigators conducted a retrospective study of 92 Chinese patients with lupus nephritis onset at or before age 18 years who presented to their health care center from 2001 to 2020. Proliferative class 3 or 4 with or without class 5 disease affected 83 children (90%).
Immunosuppression regimens changed over the 2 decades. Overall, mycophenolate was used for induction in 36% of patients and cyclophosphamide in 34%; 55% of patients received mycophenolate for maintenance. Complete and partial remission rates at 12 months were 78% and 8%, respectively. Stage 3-5 chronic kidney disease (CKD) developed in 5 patients (5.4%) and end-stage kidney disease (ESKD) in 3 patients (3.2%). Two patients died.
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Rates of survival free from advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2%, and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively, Alison Lap-tak Ma, MBBS, of Hong Kong Children’s Hospital in China, and colleagues reported in Kidney International Reports.
In multivariate analysis, ESKD requiring dialysis at presentation, nonresponse after 12 months of treatment, and multiple nephritis flares were significantly associated with 37.7-, 11.2-, and 2.6-fold increased risks for advanced CKD, ESKD, and death, respectively, the investigators reported.
Infections occurred at a rate of 2.9 episodes per 100 patient-years. The investigators urged judicious immunosuppression.
Of the cohort, osteopenia developed in 32% of patients, hypertension in 17%, short stature in 14%, and avascular necrosis in 7%.
According to Dr Ma’s team, “children with [lupus nephritis] show fairly promising long-term patient and renal survival rates when treated with long-term effective immunosuppression and measures to ensure medication adherence.”
Reference
Chan E YH, Yap D YH, Wong WT, Lai WM, Chan TM, Ma A LT. Long-term outcomes of children and adolescents with biopsy-proven childhood-onset lupus nephritis. Kidney Int Rep. Published online October 21, 2022. doi:10.1016/j.ekir.2022.10.014
