5th International Molecular Immunology and Immunogenetics Congress, İzmir, Turkey, 20 - 22 October 2022
INTRODUCTION
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.
METHOD
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.
RESULTS
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.
CONCLUSION:
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.