Monoclonal antibody targeting IFNβ for the treatment of NXP2-positive ulcerative juvenile dermatomyositis.

This report outlines the case of a 9-year-old girl with severe anti-nuclear matrix protein 2 antibody-positive juvenile dermatomyositis presenting with disease refractory to multiple traditional therapies. Treatment with an experimental monoclonal antibody inhibiting interferon beta (IFNβ) resulted in the rapid resolution of her muscle weakness and cutaneous and gastrointestinal ulcers. This case highlights the potential benefit of inhibiting IFNβ in the management of recalcitrant juvenile dermatomyositis.