Dermatomyositis is a diffuse skeletal myositis disease with a variety
of causes.The main pathological features are skeletal muscle degeneration, necrosis and lymphocyte infiltration.The onset is mainly related to humoral immune abnormalities.It is speculated that DM may be a complement-mediated microvascular disease.Myositis-specific and myositis-related antibodies can be detected in the serum.Some therapeutic drugs,such as hormones and immunosuppressants,have poor therapeutic effects.In recent years,tofacitinib citrate has been reported to be effective in the treatment of dermatomyositis.Tofacitinib is a relatively nonspecific JAK-i that affects the phosphorylation of different STAT (including STAT1 and STAT3)targets and inhibits a variety of proinflammatory cytokines.Tofacitinib has a good therapeutic effect on MDA5-positive dermatomyositis.
We report a case of MDA5-positive dermatomyositis without remission after hormone and traditional immunosuppressive therapy. Therefore,we treated the patient with prednisoneat and tofacitinib.After one month,the patient's all symptoms were relieved.however,severe cholecystitis and gangrene occurred.Burmester and Fleischmann reported that the risk of upper respiratory tract infection after tofacitinib treatment of dermatomyositis.Acute gangrenous cholecystitis occurred in our case.In addition to inflammation,gangrenous cholecystitis is mainly due to gallbladder circulation disorder,leading to bleeding and gallbladder tissue necrosis.Patients with dermatomyositis can have vasculitis, leading to tissue ischemia and necrosis.There are few reports of similar cases.There is an increased risk of infection during treatment of connective tissue diseases with tofacitinib.The infection in our case may have been caused by tofacitinib.Tofacitinib was used again two weeks after the operation.After 5 months,The patient's condition was controlled and no infection was occurring .
In recent years,there are many reports about the treatment of autoimmune diseases and infectious diseases with tofacitinib.Lee reported systemic lupus erythematosus with cholecystitis as the first manifestations.Tofacitinib is effective in the treatment of rheumatoid arthritis,ankylosing spondylitis,psoriatic arthritis,Behcet's disease, systemic vasculitis and other diseases.Zhu reported tofacitinib treatment of refractory cutaneous leukocytoclastic vasculitis.In infectious diseases,Tatiana reported that tofacitinib was effective in 320 patients with COVID-19,this is a prospective observational series.Tong's findings that tofacitinib reduced death or respiratory failure at 28 day in patients hospitalized with COVID-19 pneumonia.
However,there is an increased risk of thrombosis and infection during treatment of connective tissue diseases with tofacitinib.such as herpesvirus infection and respiratory tract infection.
In conclusion,tofacitinib is effective in the treatment of MDA5-positive dermatomyositis.However,the risk of infection which leads to dysfunction of important organs is increased.Therefore,a close follow-up should be performed during the use of tofacitinib.