B lymphocytes in systemic sclerosis: Abnormalities and therapeutic targets

The Journal of Dermatology
Ayumi Yoshizaki

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive extracellular matrix deposition in the skin and visceral organs with an autoimmune background. Although the pathogenic relationship between systemic autoimmunity and the clinical manifestations remains unknown, SSc patients have immunological abnormalities including the production of disease-specific autoantibodies. Recent studies have demonstrated that B cells play a crucial role in systemic autoimmunity and disease expression via various functions in addition to autoantibody production. Recent studies show that B cells from SSc patients demonstrate an upregulated CD19 signaling pathway, which is a crucial regulator of B-cell activation, that induces SSc-specific autoantibody production in SSc. In addition, B cells from SSc patients exhibit an overexpression of CD19. Consistently, in CD19 transgenic mice, CD19 overexpression induces SSc-specific autoantibody production. SSc patients have also intrinsic B-cell abnormalities characterized by chronic hyperreactivity of memory B cells, possibly due to CD19 overexpression. Similarly, B cells from a tight-skin mouse, a genetic model of SSc, show augmented CD19 signaling and chronic hyperreactivity. Fu...Continue Reading

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Citations

Jun 21, 2016·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Ayumi Yoshizaki
Aug 5, 2017·The Journal of Dermatology·Yanxia Chen, Jinlin Liu
Jan 15, 2020·Lupus·H A NoureldineI Uthman
Oct 1, 2017·Science Immunology·Charles F A de BourcyStephen R Quake
Dec 21, 2017·Inflammation and Regeneration·Yoshihide Asano
Apr 2, 2020·Arthritis Research & Therapy·Jakob EinhausDominik Schneidawind
May 11, 2017·Immunologic Research·Masutaka FurueTakafumi Kadono
May 1, 2017·Clinical Reviews in Allergy & Immunology·Taher E TaherRizgar A Mageed

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