Abstract
Lupus nephritis is one of the most common clinical manifestations of SLE. In childhood SLE, most of the patients complicate lupus nephritis. Severity of lupus nephritis influences not only prognosis but treatment protocol. Therefore, renal biopsy before initiation of treatment should be necessary in addition to comprehensive screening of multiple organs. Evaluation of Sjögren syndrome and anti-phospholipid syndrome is also important. Monitoring of disease activity is based on anti dsDNA antibody titer, complement (CH50, C3, C4) and erythrocyte sedimentation rate. In childhood lupus, immunosuppressive agents should be positively used to reduce dosage of steroid, increase height gain, maintain remission, and prevent relapse. In 2015, MMF was finally approved for lupus nephritis in Japan, which might improve patient's outcome and quality of life in the future.