Abstract
Patients with childhood-onset systemic lupus erythematosus （SLE） often follow a more acute and
severe clinical course compared with those with adult-onset SLE. Herein, we report a case of refractory SLE.
An 11-year-old boy was diagnosed with severe lupus nephritis （LN）, classified as IV-S（A） on histology
using the International Society of Nephrology/Renal Pathology Society classification. He was treated with
methylprednisolone pulse therapy （MPT）, mycophenolate mofetil （MMF）, and intravenous
cyclophosphamide for remission. Seven months after treatment initiation, the patient experienced a
recurrence of SLE, associated with deteriorated renal function. He was retreated with MPT, MMF,
tacrolimus, and hydroxychloroquine. A second renal biopsy was performed； he had progressed to class Ⅳ-G
（A/C） on histology with either crescent formation or segmental sclerosis or both in all glomerulus.
Therefore, we introduced immuno-adsorption plasmapheresis, as well as repeated rituximab treatment. It
took approximately 10 months to achieve normal urinary findings after the recurrence, despite a persistently
high level of serum anti-double-stranded DNA antibody. Male sex, childhood onset, and associated LN are
adverse prognostic factors of SLE. Thus, remission induction in the early stage of the disease should include
treatment with multi-target therapy, immuno-adsorption plasmapheresis and B-cell depletion in male
patients with childhood-onset SLE complicated with severe LN.
