Pediatric systemic lupus erythematosus (SLE) is a classic auto-reactive disease characterized by the presence of large amounts of autoantibodies against nuclear antigens in the blood of affected children and manifests as multisystem damage. The disease has the highest incidence in girls in Asia. The kidneys are the most commonly invaded organ in SLE, with urine tests or hyperalgesia appearing in 25-50% of children early in the course of the disease, and later progressing to significant renal damage in 80% of children, which is more common and relatively more severe than in adults, with type IV (diffuse proliferative type) accounting for 54% of cases. 1/3 of the causes of death in SLE are due to renal failure. Therefore, pediatric lupus nephritis is a common disease that seriously threatens our adolescents. Age of onset and gender: It is mostly seen in females, accounting for about 80-90% of cases, and is most common in children aged 10-14 years, with 1/3 of cases occurring in 5-10 years old and very rare in infants and children. Renal damage manifestations: not characteristic, hematuria, proteinuria, hypertension are more common: proteinuria is almost always present, with varying degrees of edema, about 50% show nephrotic syndrome; persistent microscopic hematuria is common but not yet reported alone; 40% have hypertension. About 50% have decreased renal function, which may occasionally manifest as acute renal failure. Renal tubular dysfunction is often present. Time of appearance of renal damage: may coexist with systemic symptoms; may also be the first symptom; or may appear several years after the appearance of systemic symptoms, according to statistics 60% of LN appear within 3 months of onset, 88% appear within 3 years, and 27% of renal involvement occurs 5 years or more after onset. Extra-renal manifestations: 1. rash: facial butterfly rash is common, aggravated by sunlight. 2, joint symptoms: joint muscle pain, may involve large and small joints of the extremities, showing an acute inflammatory process. 3, cardiovascular abnormalities: lesions can involve the endocardium, myocardium and pericardium, a few may have vasculitis and Raynaud’s phenomenon. 4.Respiratory system: it may manifest as interstitial pneumonia, pleurisy, pleural effusion. 5, the nervous system: mild involvement is common, such as impaired judgment, short-term memory, abnormal behavior, headache. In addition, psychosis and chorea may also be manifested, and convulsions are relatively rare. 6, gastrointestinal tract: mild gastrointestinal involvement is common, such as nausea, poor appetite, abdominal pain, liver enlargement or splenomegaly accounted for 30-40%. 7. Hematological system: anemia is most common, followed by leukopenia and thrombocytopenia. 8. Other: fever, malaise, weight loss, photosensitivity, hair loss and lymph node enlargement, etc. Treatment principles: control lupus activity, actively improve and stop kidney damage. Insist on long-term treatment, minimize the side effects of drugs, and strengthen follow-up. Commonly used drugs are: 1. Adrenocorticosteroids: Prednisone 1.5-2 mg/kg/d (total <60 mg) for 6-8 weeks, reduce the dosage according to the treatment response, gradually change to every other day (become every other day if possible), and maintain for several years when reduced to the equivalent of 10-15 mg/d or 20-30 mg/qod. Methylprednisolone shock of 1g/m2/d is given at departure or at disease outbreak for 3 days as a course of treatment, which may be repeated for 1-3 courses depending on the condition. Hormonal side effects should be noted during the long-term use of the drug. 2. Cytotoxic drugs: Combined with hormones to improve the survival rate of lupus nephritis. 3.Plasma replacement: This therapy can effectively remove antigens, antibodies and immune complexes from plasma and improve phagocytosis of reticuloendothelial system, which can be tried in diffuse proliferative lupus nephritis when hormones and immunosuppressants cannot achieve better efficacy. 4.Dialysis or transplantation: It is suitable for patients with renal failure. In addition, there are some new therapies, such as total body lymph node irradiation, intravenous injection of immunoglobulin, monoclonal antibodies, etc.