Relationship Interferon Signalization and Disease Activation in Patients with Juvenile Scleroderma.

Köse H., Şimşek A., Kızmaz M. A., Bozkurt T., Öztürk F., Budak F., ...More

5th International Molecular Immunology and Immunogenetics Congress, İzmir, Turkey, 20 - 22 October 2022

  • Publication Type: Conference Paper / Summary Text
  • City: İzmir
  • Country: Turkey
  • Bursa Uludag University Affiliated: Yes



Juvenile dermatomyositis (JDM) is an inflammatory myopathy characterized by skin involvement, vasculitis, and progressive muscle weakness. Increased interferon signaling is thought to play a role in the pathogenesis of JDM and is associated with disease severity and activation, as well as the development of calcinosis. Juvenile scleroderma (JSc) is a heterogeneous group of diseases associated with sclerotic skin lesions, grouped together as systemic sclerosis and localized scleroderma (morphea). Although JSc has a different skin involvement than JDM, some similarities in their pathogenesis can be identified. The aim of this study is to measure the levels of cytokines and chemokines involved in interferon signaling in patients with JDM and JSc and to determine their correlation with disease severity.


Thirty-one JSc, 5 JDM, and 13 healthy controls were included in the study. Patients with morphea were scored according to the LoSCAT (activity index) and LoSDI (damage index) indices. Cytokines and chemokines involved in interferon gene signaling (IL -8, IL -1,IP-10, MCP1, TNF-α, CXCL-10, IFN-α, IFN-β, IFN-γ) were measured by ELISA.


The ratio of female to male patients was 24/12, the median age was 14 years (min 4, max 18), and the median follow-up time was 36 months (min 12, max 108).

It was found that IL-8, IFN-β, IP-10, and MCP-1 were significantly decreased in patients with JSc compared with the healthy control group. TNF-α and IFN-γ were found to tend to decrease in patients with JSc, whereas IFN-α tended to increase in patients with JDM.
The correlation of LoSCAT and LoSDI indices with cytokine and chemokine levels in patients with JSc is summarized in Figure 1.




The results suggest that interferon signaling may be impaired in patients with JDM and JSc, and that cytokines whose significant changes were observed in this study may play a key role in monitoring disease activity.