Systemic sclerosis (SSc) is a connective tissue disease, the pathogenesis of which is thought to involve interleukin-6 (IL-6), an inflammatory cytokine. This is based on findings of its concentration in patient serum, the results of an IL-6 suppression experiment in an animal model, and the results of a pilot study using IL-6 receptor antibody. However, it appears that a number of factors are involved in the pathology of SSc depending on the state of disease progression. In addition, the degree of involvement of IL-6 differs depending on the difference of organs within particular severe symptoms. Based on the findings from measurements of patient serum, the influence of IL-6 on the pathogenesis of SSc is greater in patients at a relatively early phase of the disease and in patients with lung lesions. Interleukin-13 (IL-13) is one of pro-fibrotic factors, and it is afraid that SSc patients with higher IL-13 have already lost the influence of IL-6. Therefore, although a clinical trial using the anti-IL-6 receptor antibody tocilizumab is underway, it is important to recognize the state of SSc patients prior to selecting treatment.