Korean J Med > Volume 73(3); 2007 > Article
The Korean Journal of Medicine 2007;73(3):1076-1080.
A case of systemic sclerosis with good response to rituximab
Yun Jung Park, Su-Yeon Lee, Jae Hyoung Cho, Seung Hyun Oh, Jennifer Lee, Hyun-Sook Kim, Wan-Uk Kim
경희대학교 의과대학 순환기내과학교실
증례 : Rituximab 치료 후 호전된 전신 피부 경화증 1예
박윤정&#;이수연&#;조재형&#;오승현&#;이주하&#;김현숙&#;김완욱, Su-Yeon Lee, Jae Hyoung Cho, Seung Hyun Oh, Jennifer Lee, Hyun-Sook Kim, Wan-Uk Kim
Abstract
Systemic sclerosis (SSc) is a connective tissue disease characterized by tissue fibrosis. Classically the pathogenesis of SSc involves three main disease processes including mainly T-cell mediated upregulated autoimmunity, excess fibrosis due to increased fibroblast activity and proliferative obliterating vasculopathy. However, recent studies suggest that SSc patients have intrinsic B cell abnormalities characterized by decreased but activated memory B cells. Augmented cytokine production, by B cells, is a potential candidate for the induction of skin sclerosis. In addition, B cells may influence tissue fibrosis by regulating T cell activation and cytokine production through their antigen-presenting and co-stimulatory activities. Current data obtained from studies on B-cell ablation therapy using rituximab for autoimmune diseases show good tolerability and sustained improvement in disease symptoms. We treated a patient with refractory SSc who showed improvement of the skin sclerosis after rituximab therapy.(Korean J Med 73:S1076-S1080, 2007)
Key Words: Systemic sclerosis, Rituximab


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