A French team has confirmed the link between the activation of interferon 1 (IFN) and the onset of dermatomyositis, and have demonstrated the efficacy of an interferon 1 inhibitor in 4 people with this disease.
People with dermatomyositis have abnormal levels of IFN in the blood and muscles. A link between the activation of IFN and dermatomyositis has already been identified, but its exact mechanism is unclear.
In an article published in June 2018, a French team, supported by the AFM-Telethon, showed that the activation of IFN in muscle or skin cell cultures reproduced the main characteristics of dermatomyositis, such as muscle atrophy or skin lesions. Treatment with ruxolitinib, a compound already used in the treatment of lupus erythematosus (another autoimmune disease), inhibits the pathogenic effects of IFN-I in the cellular models studied.
The administration of ruxolitinib for 3 months to 4 people with refractory dermatomyositis led to rapid clinical improvement: decreased skin lesions and improved muscle strength.
