关键词: 幼年皮肌炎, 治疗, 糖皮质激素, 免疫抑制剂, 生物制剂, 肌炎特异性抗体

Abstract:

Juvenile dermatomyositis (JDM) is an autoimmune inflammatory myopathy in childhood that can affect the whole body, mainly the skin and proximal muscles. It can also affect vital organs (such as: lungs, joints, and gastrointestinal tract etc), often accompanied by the presence of specific antibodies. At present, the treatment of JDM is very difficult. The first-line drugs are glucocorticoids and methotrexate. For patients with poor therapeutic effects, some second-line or third-line drugs may play a supportive role. In refractory patients, intravenous injection of immunoglobulin or cyclophosphamide may be effective, while biologics and small molecule targeted drugs are gradually used in clinical practice. In addition to glucocorticoids, the choice of drugs often requires a combination of clinical symptoms, serum specific antibodies, pathological results and response to initial treatment, and continuous adjustments during the treatment process. The purpose of this review is to provide an overview the drugs currently used in the treatment of JDM, observation of therapeutic effects and strategies for drug selection, and to provide ideas for clinical treatment.

Key words: juvenile dermatomyositis, treatment, glucocorticoids, immunosuppressants, biological agents, myositis specific antibody