Understanding, monitoring, and predicting interferon-driven inflammation in juvenile dermatomyositis: Towards personalised treatment

Juvenile dermatomyositis (JDM) is a rare autoimmune disease in which children develop inflammation of the muscles and skin. This can lead to severe muscle weakness and difficulties with walking, swallowing, or even breathing. Unfortunately, current treatments are not effective enough for around half of children with JDM. They continue to have active disease or experience flare-ups. In addition, both the disease itself and long-term treatment with prednisone can cause lasting damage and serious side effects.

In this thesis, Saskia Veldkamp investigated how treatment for children with JDM can become more personalised and effective. Her research focused on biomarkers: proteins in the blood that provide information about disease activity. The findings show that several immune-related biomarkers, including Galectin-9, CXCL10, and Siglec-1, can accurately reflect disease activity. Galectin-9 and CXCL10 were found to increase already months before a flare occurs, which may help doctors intervene earlier or more confidently reduce medication when disease activity is low.

The research also provides new insights into the role of interferon, a signalling protein involved in the immune response, in JDM. A laboratory model showed that the biomarker Siglec-1 can be used to measure interferon activity and assess the effects of new treatments called JAK inhibitors. The results also point towards an important role for the subtype interferon-beta in JDM.

These findings bring personalised treatment for children with JDM one step closer. By improving the ability to predict how the disease will develop and tailoring treatment accordingly, future care may help prevent lasting damage and reduce unnecessary medication side effects.