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 |
박윤정이수연조재형오승현이주하김현숙김완욱, Su-Yeon Lee, Jae Hyoung Cho, Seung Hyun Oh, Jennifer Lee, Hyun-Sook Kim, Wan-Uk Kim |
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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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