Dermatomyositis is a heterogeneous disease characterized by involvement of the proximal muscles of the extremities. Some patients have skin, lung and other systems involved. The clinical manifestations of dermatomyositis are varied and vary from person to person, so the treatment should follow the principle of individualization. At present, glucocorticoid combined with immunosuppressants (including methotrexate, azathioprine, cyclosporine, tacrolimus, mycophoranate, cyclophosphamide, etc.) is the first choice for the treatment of dermatomyositis. Some patients have treatment failure or intolerance to the above treatment regimens, which is called refractory dermatomyositis. There has not been a large-scale study to explore the treatment regimens. Abatacept is a CTLA-4 fusion protein that inhibits T cell activation by competitively binding to CD80/CD86 and blocking the second signal. To date, Abatacept has been approved by the FDA for the treatment of three types of rheumatoid immune diseases (refractory rheumatoid arthritis, juvenile idiopathic arthritis, and active psoriatic arthritis). The international consensus of experts recommends abacepil as a second-line regimen for the treatment of refractory dermatomyositis based on the evidence of case reports. However, to date, there is no relevant report or clinical study registration information of Abatacept in the treatment of dermatomyositis. The purpose of this study was to prospectively observe the efficacy and safety of Abatacept in the treatment of refractory dermatomyositis through a controlled before and after study.
Dermatomyositis is a heterogeneous disease characterized by involvement of the proximal muscles of the extremities. Some patients have treatment failure or intolerance to the above treatment regimens, which is called refractory dermatomyositis. Abatacept has been approved by the FDA for the treatment of three types of rheumatoid immune diseases, and the international consensus of experts recommends abacepil as a second-line regimen for the treatment of refractory dermatomyositis based on the evidence of case reports.