Even if you are a "little butterfly", you must try your best to fly

Author: Liu Xuanyi Beijing Children's Hospital Affiliated to Capital Medical University

Reviewer: Li Caifeng, Chief Physician, Beijing Children's Hospital, Capital Medical University

Childhood systemic lupus erythematosus (cSLE) is an autoimmune disease that invades multiple systems and organs. Children have multiple autoantibodies represented by antinuclear antibodies. Children are not smaller versions of adults. There are some similarities and differences between cSLE and adult SLE.

In terms of the incidence, the prevalence of lupus in China is 30-70/100,000, and cSLE accounts for 10%-20% of the total number of SLE cases. The peak incidence is between 12 and 14 years old, that is, it is highly prevalent during adolescence. The male-female ratio of the disease varies in each age group. In preschool SLE (<6 years old), the male-female ratio is roughly equal (1:1). In school-age children and before and after adolescence, this ratio is 1:4-5, and girls are more common; after adolescence, the male-female ratio is 1:9-10, showing a typical distribution. The cause of cSLE is unclear, and current studies have shown that it is related to the interaction between race, genetics, immune regulation and environmental factors.

The clinical manifestations of cSLE are diverse, and the first symptoms vary. Most children will have non-specific symptoms such as fever, lack of appetite, and fatigue. About half of the children may develop typical butterfly erythema, so we often call cSLE children "butterfly babies". Some children will experience hair loss, oral ulcers, and sunlight allergy. Musculoskeletal involvement can cause myalgia and joint pain; cardiac involvement can cause chest pain and myocarditis; lung involvement can cause dyspnea, cough, and chest pain; kidney involvement can show abnormal urine tests; digestive tract involvement can cause nausea, vomiting, abdominal pain, and diarrhea; nervous system involvement can cause headaches, impaired consciousness, and convulsions. Pediatric rheumatologists can evaluate the involvement of various organs through the clinical manifestations of children and the results of auxiliary examinations. However, compared with patients with adult onset, cSLE is more severe, with higher disease activity and drug burden, more severe organ damage, and a higher incidence of important organs such as kidney, cardiovascular, and neuropsychiatric diseases, resulting in higher disease-related disability and mortality.

Currently, the same diagnostic criteria are used for children and adults. Rheumatologists will assess the disease activity of children and provide individualized treatment according to the different levels of activity. The treatment goals for children with cSLE are to control clinical symptoms, improve quality of life, reduce organ damage, and reduce relapse and mortality rates.

Children and adolescents use the same drugs as adult SLE patients during treatment, including the longer-term use of glucocorticoids and immunosuppressants, but the dosage for children is calculated based on kilograms of body weight or body surface area. In addition, due to the characteristics of children's diseases, more active treatment is usually required to achieve the goal of controlling the disease. The figure below is a schematic diagram of the treatment goals and effects of lupus in children.

Copyright images are not authorized for reproduction

Therefore, even a "little butterfly" must fly, and pediatric rheumatologists will work together with children with cSLE!